PMC:1448208 / 2104-7183 JSON TXT

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Id	Subject	Object	Predicate	Lexical cue
T3318	78-88	NN	denotes	deficiency
T3319	89-91	IN	denotes	of
T3320	92-100	VBN	denotes	branched
T3321	101-106	NN	denotes	chain
T3322	100-101	HYPH	denotes	-
T3323	117-130	NN	denotes	dehydrogenase
T3324	107-111	NN	denotes	keto
T3325	112-116	NN	denotes	acid
T3326	131-132	-LRB-	denotes	(
T3327	132-137	NN	denotes	BCKDH
T3328	137-138	-RRB-	denotes	)
T3329	138-140	,	denotes	,
T3330	140-141	DT	denotes	a
T3331	168-175	NN	denotes	complex
T3332	142-155	JJ	denotes	mitochondrial
T3333	156-167	NN	denotes	multienzyme
T3334	176-187	JJ	denotes	responsible
T3335	188-191	IN	denotes	for
T3336	192-195	DT	denotes	the
T3337	206-221	NN	denotes	decarboxylation
T3338	196-205	JJ	denotes	oxidative
T3339	222-224	IN	denotes	of
T3340	225-233	VBN	denotes	branched
T3341	234-239	NN	denotes	chain
T3342	233-234	HYPH	denotes	-
T3343	245-250	NNS	denotes	acids
T3344	240-244	NN	denotes	keto
T3345	251-258	VBN	denotes	derived
T3346	259-263	IN	denotes	from
T3347	264-272	VBN	denotes	branched
T3348	273-278	NN	denotes	chain
T3349	272-273	HYPH	denotes	-
T3350	285-290	NNS	denotes	acids
T3351	279-284	NN	denotes	amino
T3352	291-292	-LRB-	denotes	(
T3353	292-296	NN	denotes	BCAA
T3354	296-297	-RRB-	denotes	)
T3355	297-299	,	denotes	,
T3356	299-306	NN	denotes	leucine
T3357	306-308	,	denotes	,
T3358	308-318	NN	denotes	isoleucine
T3359	319-322	CC	denotes	and
T3360	323-329	NN	denotes	valine
T3361	330-331	-LRB-	denotes	(
T3362	343-346	VB	denotes	see
T3363	331-334	IN	denotes	for
T3364	335-341	NN	denotes	review
T3365	341-343	,	denotes	,
T3366	346-348	:	denotes	:
T3367	348-349	-LRB-	denotes	[
T3368	349-350	CD	denotes	1
T3369	350-351	-RRB-	denotes	]
T3370	351-352	-RRB-	denotes	)
T3371	352-353	.	denotes	.
T3372	353-497	sentence	denotes	Patients with MSUD, depending on the mutation, show variable degrees of enzyme deficiency leading to several different clinical phenotypes [1].
T3373	354-362	NNS	denotes	Patients
T3374	401-405	VBP	denotes	show
T3375	363-367	IN	denotes	with
T3376	368-372	NN	denotes	MSUD
T3377	372-374	,	denotes	,
T3378	374-383	VBG	denotes	depending
T3379	384-386	IN	denotes	on
T3380	387-390	DT	denotes	the
T3381	391-399	NN	denotes	mutation
T3382	399-401	,	denotes	,
T3383	406-414	JJ	denotes	variable
T3384	415-422	NNS	denotes	degrees
T3385	423-425	IN	denotes	of
T3386	426-432	NN	denotes	enzyme
T3387	433-443	NN	denotes	deficiency
T3388	444-451	VBG	denotes	leading
T3389	452-454	IN	denotes	to
T3390	455-462	JJ	denotes	several
T3391	482-492	NNS	denotes	phenotypes
T3392	463-472	JJ	denotes	different
T3393	473-481	JJ	denotes	clinical
T3394	493-494	-LRB-	denotes	[
T3395	494-495	CD	denotes	1
T3396	495-496	-RRB-	denotes	]
T3397	496-497	.	denotes	.
T3398	497-625	sentence	denotes	Approximately 75% of MSUD patients have the classic form of the disease with BCKDH activity in the range of 0–2% of normal [1].
T3399	498-511	RB	denotes	Approximately
T3400	512-514	CD	denotes	75
T3401	514-515	NN	denotes	%
T3402	533-537	VBP	denotes	have
T3403	516-518	IN	denotes	of
T3404	519-523	NN	denotes	MSUD
T3405	524-532	NNS	denotes	patients
T3406	538-541	DT	denotes	the
T3407	550-554	NN	denotes	form
T3408	542-549	JJ	denotes	classic
T3409	555-557	IN	denotes	of
T3410	558-561	DT	denotes	the
T3411	562-569	NN	denotes	disease
T3412	570-574	IN	denotes	with
T3413	575-580	NN	denotes	BCKDH
T3414	581-589	NN	denotes	activity
T3415	590-592	IN	denotes	in
T3416	593-596	DT	denotes	the
T3417	597-602	NN	denotes	range
T3418	603-605	IN	denotes	of
T3419	606-607	CD	denotes	0
T3420	608-609	CD	denotes	2
T3421	607-608	SYM	denotes	–
T3422	609-610	NN	denotes	%
T3423	611-613	IN	denotes	of
T3424	614-620	JJ	denotes	normal
T3425	621-622	-LRB-	denotes	[
T3426	622-623	CD	denotes	1
T3427	623-624	-RRB-	denotes	]
T3428	624-625	.	denotes	.
T3429	625-714	sentence	denotes	These patients show markedly elevated levels of BCAA in blood and other body fluids [1].
T3430	626-631	DT	denotes	These
T3431	632-640	NNS	denotes	patients
T3432	641-645	VBP	denotes	show
T3433	646-654	RB	denotes	markedly
T3434	655-663	VBN	denotes	elevated
T3435	664-670	NNS	denotes	levels
T3436	671-673	IN	denotes	of
T3437	674-678	NN	denotes	BCAA
T3438	679-681	IN	denotes	in
T3439	682-687	NN	denotes	blood
T3440	688-691	CC	denotes	and
T3441	692-697	JJ	denotes	other
T3442	703-709	NNS	denotes	fluids
T3443	698-702	NN	denotes	body
T3444	710-711	-LRB-	denotes	[
T3445	711-712	CD	denotes	1
T3446	712-713	-RRB-	denotes	]
T3447	713-714	.	denotes	.
T3448	714-781	sentence	denotes	Besides the classic form, there are other variants of the disease.
T3449	715-722	IN	denotes	Besides
T3450	747-750	VBP	denotes	are
T3451	723-726	DT	denotes	the
T3452	735-739	NN	denotes	form
T3453	727-734	JJ	denotes	classic
T3454	739-741	,	denotes	,
T3455	741-746	EX	denotes	there
T3456	751-756	JJ	denotes	other
T3457	757-765	NNS	denotes	variants
T3458	766-768	IN	denotes	of
T3459	769-772	DT	denotes	the
T3460	773-780	NN	denotes	disease
T3461	780-781	.	denotes	.
T3462	781-885	sentence	denotes	Patients with the intermediate form of the disease show BCKDH activity in the range of 3–30% of normal.
T3463	782-790	NNS	denotes	Patients
T3464	833-837	VBP	denotes	show
T3465	791-795	IN	denotes	with
T3466	796-799	DT	denotes	the
T3467	813-817	NN	denotes	form
T3468	800-812	JJ	denotes	intermediate
T3469	818-820	IN	denotes	of
T3470	821-824	DT	denotes	the
T3471	825-832	NN	denotes	disease
T3472	838-843	NN	denotes	BCKDH
T3473	844-852	NN	denotes	activity
T3474	853-855	IN	denotes	in
T3475	856-859	DT	denotes	the
T3476	860-865	NN	denotes	range
T3477	866-868	IN	denotes	of
T3478	869-870	CD	denotes	3
T3479	871-873	CD	denotes	30
T3480	870-871	SYM	denotes	–
T3481	873-874	NN	denotes	%
T3482	875-877	IN	denotes	of
T3483	878-884	JJ	denotes	normal
T3484	884-885	.	denotes	.
T3485	885-995	sentence	denotes	In such patients the onset of the disease is delayed, but there are persistently elevated levels of BCAA [1].
T3486	886-888	IN	denotes	In
T3487	931-938	VBN	denotes	delayed
T3488	889-893	JJ	denotes	such
T3489	894-902	NNS	denotes	patients
T3490	903-906	DT	denotes	the
T3491	907-912	NN	denotes	onset
T3492	913-915	IN	denotes	of
T3493	916-919	DT	denotes	the
T3494	920-927	NN	denotes	disease
T3495	928-930	VBZ	denotes	is
T3496	938-940	,	denotes	,
T3497	940-943	CC	denotes	but
T3498	944-949	EX	denotes	there
T3499	950-953	VBP	denotes	are
T3500	954-966	RB	denotes	persistently
T3501	967-975	VBN	denotes	elevated
T3502	976-982	NNS	denotes	levels
T3503	983-985	IN	denotes	of
T3504	986-990	NN	denotes	BCAA
T3505	991-992	-LRB-	denotes	[
T3506	992-993	CD	denotes	1
T3507	993-994	-RRB-	denotes	]
T3508	994-995	.	denotes	.
T3509	995-1153	sentence	denotes	Patients with the intermittent form of MSUD show BCKDH activity in the range of 5–20% and during the asymptomatic phase the blood BCAA levels are normal [1].
T3510	996-1004	NNS	denotes	Patients
T3511	1040-1044	VBP	denotes	show
T3512	1005-1009	IN	denotes	with
T3513	1010-1013	DT	denotes	the
T3514	1027-1031	NN	denotes	form
T3515	1014-1026	JJ	denotes	intermittent
T3516	1032-1034	IN	denotes	of
T3517	1035-1039	NN	denotes	MSUD
T3518	1045-1050	NN	denotes	BCKDH
T3519	1051-1059	NN	denotes	activity
T3520	1060-1062	IN	denotes	in
T3521	1063-1066	DT	denotes	the
T3522	1067-1072	NN	denotes	range
T3523	1073-1075	IN	denotes	of
T3524	1076-1077	CD	denotes	5
T3525	1078-1080	CD	denotes	20
T3526	1077-1078	SYM	denotes	–
T3527	1080-1081	NN	denotes	%
T3528	1082-1085	CC	denotes	and
T3529	1086-1092	IN	denotes	during
T3530	1138-1141	VBP	denotes	are
T3531	1093-1096	DT	denotes	the
T3532	1110-1115	NN	denotes	phase
T3533	1097-1109	JJ	denotes	asymptomatic
T3534	1116-1119	DT	denotes	the
T3535	1131-1137	NNS	denotes	levels
T3536	1120-1125	NN	denotes	blood
T3537	1126-1130	NN	denotes	BCAA
T3538	1142-1148	JJ	denotes	normal
T3539	1149-1150	-LRB-	denotes	[
T3540	1150-1151	CD	denotes	1
T3541	1151-1152	-RRB-	denotes	]
T3542	1152-1153	.	denotes	.
T3543	1153-1352	sentence	denotes	The overall incidence of MSUD in the general population is 1:185,000 [1], and in certain population groups, such as Mennonites of Pennsylvania, the incidence is estimated to be as high as 1:176 [2].
T3544	1154-1157	DT	denotes	The
T3545	1166-1175	NN	denotes	incidence
T3546	1158-1165	JJ	denotes	overall
T3547	1210-1212	VBZ	denotes	is
T3548	1176-1178	IN	denotes	of
T3549	1179-1183	NN	denotes	MSUD
T3550	1184-1186	IN	denotes	in
T3551	1187-1190	DT	denotes	the
T3552	1199-1209	NN	denotes	population
T3553	1191-1198	JJ	denotes	general
T3554	1213-1214	CD	denotes	1
T3555	1215-1222	CD	denotes	185,000
T3556	1214-1215	SYM	denotes	:
T3557	1223-1224	-LRB-	denotes	[
T3558	1224-1225	CD	denotes	1
T3559	1225-1226	-RRB-	denotes	]
T3560	1226-1228	,	denotes	,
T3561	1228-1231	CC	denotes	and
T3562	1232-1234	IN	denotes	in
T3563	1315-1324	VBN	denotes	estimated
T3564	1235-1242	JJ	denotes	certain
T3565	1254-1260	NNS	denotes	groups
T3566	1243-1253	NN	denotes	population
T3567	1260-1262	,	denotes	,
T3568	1262-1266	JJ	denotes	such
T3569	1267-1269	IN	denotes	as
T3570	1270-1280	NNS	denotes	Mennonites
T3571	1281-1283	IN	denotes	of
T3572	1284-1296	NNP	denotes	Pennsylvania
T3573	1296-1298	,	denotes	,
T3574	1298-1301	DT	denotes	the
T3575	1302-1311	NN	denotes	incidence
T3576	1312-1314	VBZ	denotes	is
T3577	1325-1327	TO	denotes	to
T3578	1328-1330	VB	denotes	be
T3579	1331-1333	IN	denotes	as
T3580	1334-1338	JJ	denotes	high
T3581	1339-1341	IN	denotes	as
T3582	1342-1343	CD	denotes	1
T3583	1344-1347	CD	denotes	176
T3584	1343-1344	SYM	denotes	:
T3585	1348-1349	-LRB-	denotes	[
T3586	1349-1350	CD	denotes	2
T3587	1350-1351	-RRB-	denotes	]
T3588	1351-1352	.	denotes	.
T3589	1352-1536	sentence	denotes	The BCKDH complex, the deficient enzyme in MSUD, consists of three catalytic proteins, a decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoyl dehydrogenase (E3).
T3590	1353-1356	DT	denotes	The
T3591	1363-1370	NN	denotes	complex
T3592	1357-1362	NN	denotes	BCKDH
T3593	1402-1410	VBZ	denotes	consists
T3594	1370-1372	,	denotes	,
T3595	1372-1375	DT	denotes	the
T3596	1386-1392	NN	denotes	enzyme
T3597	1376-1385	JJ	denotes	deficient
T3598	1393-1395	IN	denotes	in
T3599	1396-1400	NN	denotes	MSUD
T3600	1400-1402	,	denotes	,
T3601	1411-1413	IN	denotes	of
T3602	1414-1419	CD	denotes	three
T3603	1430-1438	NN	denotes	proteins
T3604	1420-1429	JJ	denotes	catalytic
T3605	1438-1440	,	denotes	,
T3606	1440-1441	DT	denotes	a
T3607	1442-1455	NN	denotes	decarboxylase
T3608	1456-1457	-LRB-	denotes	(
T3609	1457-1459	NN	denotes	E1
T3610	1459-1460	-RRB-	denotes	)
T3611	1460-1462	,	denotes	,
T3612	1462-1463	DT	denotes	a
T3613	1478-1490	NN	denotes	transacylase
T3614	1464-1477	NN	denotes	dihydrolipoyl
T3615	1491-1492	-LRB-	denotes	(
T3616	1492-1494	NN	denotes	E2
T3617	1494-1495	-RRB-	denotes	)
T3618	1495-1497	,	denotes	,
T3619	1497-1500	CC	denotes	and
T3620	1501-1502	DT	denotes	a
T3621	1517-1530	NN	denotes	dehydrogenase
T3622	1503-1516	NN	denotes	dihydrolipoyl
T3623	1531-1532	-LRB-	denotes	(
T3624	1532-1534	NN	denotes	E3
T3625	1534-1535	-RRB-	denotes	)
T3626	1535-1536	.	denotes	.
T3627	1536-1615	sentence	denotes	The E1 component is a heterotetramer composed of two α and two β subunits [3].
T3628	1537-1540	DT	denotes	The
T3629	1544-1553	NN	denotes	component
T3630	1541-1543	NN	denotes	E1
T3631	1554-1556	VBZ	denotes	is
T3632	1557-1558	DT	denotes	a
T3633	1559-1573	NN	denotes	heterotetramer
T3634	1574-1582	VBN	denotes	composed
T3635	1583-1585	IN	denotes	of
T3636	1586-1589	CD	denotes	two
T3637	1590-1591	NN	denotes	α
T3638	1602-1610	NNS	denotes	subunits
T3639	1592-1595	CC	denotes	and
T3640	1596-1599	CD	denotes	two
T3641	1600-1601	NN	denotes	β
T3642	1611-1612	-LRB-	denotes	[
T3643	1612-1613	CD	denotes	3
T3644	1613-1614	-RRB-	denotes	]
T3645	1614-1615	.	denotes	.
T3646	1615-1784	sentence	denotes	The E1 and E2 components are specific to BCKDH, whereas E3 is also used by pyruvate and α-ketoglutarate dehydrogenase complexes [3] and the glycine cleavage system [4].
T3647	1616-1619	DT	denotes	The
T3648	1630-1640	NNS	denotes	components
T3649	1620-1622	NN	denotes	E1
T3650	1623-1626	CC	denotes	and
T3651	1627-1629	NN	denotes	E2
T3652	1641-1644	VBP	denotes	are
T3653	1645-1653	JJ	denotes	specific
T3654	1654-1656	IN	denotes	to
T3655	1657-1662	NN	denotes	BCKDH
T3656	1662-1664	,	denotes	,
T3657	1664-1671	IN	denotes	whereas
T3658	1683-1687	VBN	denotes	used
T3659	1672-1674	NN	denotes	E3
T3660	1675-1677	VBZ	denotes	is
T3661	1678-1682	RB	denotes	also
T3662	1688-1690	IN	denotes	by
T3663	1691-1699	NN	denotes	pyruvate
T3664	1734-1743	NNS	denotes	complexes
T3665	1700-1703	CC	denotes	and
T3666	1704-1705	NN	denotes	α
T3667	1706-1719	NN	denotes	ketoglutarate
T3668	1705-1706	HYPH	denotes	-
T3669	1720-1733	NN	denotes	dehydrogenase
T3670	1744-1745	-LRB-	denotes	[
T3671	1745-1746	CD	denotes	3
T3672	1746-1747	-RRB-	denotes	]
T3673	1748-1751	CC	denotes	and
T3674	1752-1755	DT	denotes	the
T3675	1773-1779	NN	denotes	system
T3676	1756-1763	NN	denotes	glycine
T3677	1764-1772	NN	denotes	cleavage
T3678	1780-1781	-LRB-	denotes	[
T3679	1781-1782	CD	denotes	4
T3680	1782-1783	-RRB-	denotes	]
T3681	1783-1784	.	denotes	.
T3682	1784-2027	sentence	denotes	BCKDH is also associated with two regulatory proteins, a specific kinase and a phosphatase which regulate the activity of this enzyme through a phosphorylation (inactivation) and dephosphorylation (activation) cycle of the E1 α subunit [5,6].
T3683	1785-1790	NN	denotes	BCKDH
T3684	1799-1809	VBN	denotes	associated
T3685	1791-1793	VBZ	denotes	is
T3686	1794-1798	RB	denotes	also
T3687	1810-1814	IN	denotes	with
T3688	1815-1818	CD	denotes	two
T3689	1830-1838	NN	denotes	proteins
T3690	1819-1829	JJ	denotes	regulatory
T3691	1838-1840	,	denotes	,
T3692	1840-1841	DT	denotes	a
T3693	1851-1857	NN	denotes	kinase
T3694	1842-1850	JJ	denotes	specific
T3695	1858-1861	CC	denotes	and
T3696	1862-1863	DT	denotes	a
T3697	1864-1875	NN	denotes	phosphatase
T3698	1876-1881	WDT	denotes	which
T3699	1882-1890	VBP	denotes	regulate
T3700	1891-1894	DT	denotes	the
T3701	1895-1903	NN	denotes	activity
T3702	1904-1906	IN	denotes	of
T3703	1907-1911	DT	denotes	this
T3704	1912-1918	NN	denotes	enzyme
T3705	1919-1926	IN	denotes	through
T3706	1927-1928	DT	denotes	a
T3707	1995-2000	NN	denotes	cycle
T3708	1929-1944	NN	denotes	phosphorylation
T3709	1945-1946	-LRB-	denotes	(
T3710	1946-1958	NN	denotes	inactivation
T3711	1958-1959	-RRB-	denotes	)
T3712	1960-1963	CC	denotes	and
T3713	1964-1981	NN	denotes	dephosphorylation
T3714	1982-1983	-LRB-	denotes	(
T3715	1983-1993	NN	denotes	activation
T3716	1993-1994	-RRB-	denotes	)
T3717	2001-2003	IN	denotes	of
T3718	2004-2007	DT	denotes	the
T3719	2013-2020	NN	denotes	subunit
T3720	2008-2010	NN	denotes	E1
T3721	2011-2012	NN	denotes	α
T3722	2021-2022	-LRB-	denotes	[
T3723	2024-2025	CD	denotes	6
T3724	2022-2023	CD	denotes	5
T3725	2023-2024	,	denotes	,
T3726	2025-2026	-RRB-	denotes	]
T3727	2026-2027	.	denotes	.
T3728	2027-2195	sentence	denotes	Mutations in the genes of the E1 and E2 subunits of BCKDH have been described, however, the majority of MSUD mutations identified thus far are in the E2 subunit [1,7].
T3729	2028-2037	NNS	denotes	Mutations
T3730	2096-2105	VBN	denotes	described
T3731	2038-2040	IN	denotes	in
T3732	2041-2044	DT	denotes	the
T3733	2045-2050	NNS	denotes	genes
T3734	2051-2053	IN	denotes	of
T3735	2054-2057	DT	denotes	the
T3736	2068-2076	NNS	denotes	subunits
T3737	2058-2060	NN	denotes	E1
T3738	2061-2064	CC	denotes	and
T3739	2065-2067	NN	denotes	E2
T3740	2077-2079	IN	denotes	of
T3741	2080-2085	NN	denotes	BCKDH
T3742	2086-2090	VBP	denotes	have
T3743	2091-2095	VBN	denotes	been
T3744	2167-2170	VBP	denotes	are
T3745	2105-2107	,	denotes	,
T3746	2107-2114	RB	denotes	however
T3747	2114-2116	,	denotes	,
T3748	2116-2119	DT	denotes	the
T3749	2120-2128	NN	denotes	majority
T3750	2129-2131	IN	denotes	of
T3751	2132-2136	NN	denotes	MSUD
T3752	2137-2146	NNS	denotes	mutations
T3753	2147-2157	VBN	denotes	identified
T3754	2158-2162	RB	denotes	thus
T3755	2163-2166	RB	denotes	far
T3756	2171-2173	IN	denotes	in
T3757	2174-2177	DT	denotes	the
T3758	2181-2188	NN	denotes	subunit
T3759	2178-2180	NN	denotes	E2
T3760	2189-2190	-LRB-	denotes	[
T3761	2192-2193	CD	denotes	7
T3762	2190-2191	CD	denotes	1
T3763	2191-2192	,	denotes	,
T3764	2193-2194	-RRB-	denotes	]
T3765	2194-2195	.	denotes	.
T3766	2195-2302	sentence	denotes	To date, cases of MSUD have not been associated with defects in the regulatory kinase and phosphatase [1].
T3767	2196-2198	IN	denotes	To
T3768	2233-2243	VBN	denotes	associated
T3769	2199-2203	NN	denotes	date
T3770	2203-2205	,	denotes	,
T3771	2205-2210	NNS	denotes	cases
T3772	2211-2213	IN	denotes	of
T3773	2214-2218	NN	denotes	MSUD
T3774	2219-2223	VBP	denotes	have
T3775	2224-2227	RB	denotes	not
T3776	2228-2232	VBN	denotes	been
T3777	2244-2248	IN	denotes	with
T3778	2249-2256	NNS	denotes	defects
T3779	2257-2259	IN	denotes	in
T3780	2260-2263	DT	denotes	the
T3781	2275-2281	NN	denotes	kinase
T3782	2264-2274	JJ	denotes	regulatory
T3783	2282-2285	CC	denotes	and
T3784	2286-2297	NN	denotes	phosphatase
T3785	2298-2299	-LRB-	denotes	[
T3786	2299-2300	CD	denotes	1
T3787	2300-2301	-RRB-	denotes	]
T3788	2301-2302	.	denotes	.
T3789	2302-2412	sentence	denotes	Current management of MSUD patients relies on a strict lifelong dietary restriction of protein or BCAA [1,8].
T3790	2303-2310	JJ	denotes	Current
T3791	2311-2321	NN	denotes	management
T3792	2339-2345	VBZ	denotes	relies
T3793	2322-2324	IN	denotes	of
T3794	2325-2329	NN	denotes	MSUD
T3795	2330-2338	NNS	denotes	patients
T3796	2346-2348	IN	denotes	on
T3797	2349-2350	DT	denotes	a
T3798	2375-2386	NN	denotes	restriction
T3799	2351-2357	JJ	denotes	strict
T3800	2358-2366	JJ	denotes	lifelong
T3801	2367-2374	JJ	denotes	dietary
T3802	2387-2389	IN	denotes	of
T3803	2390-2397	NN	denotes	protein
T3804	2398-2400	CC	denotes	or
T3805	2401-2405	NN	denotes	BCAA
T3806	2406-2407	-LRB-	denotes	[
T3807	2409-2410	CD	denotes	8
T3808	2407-2408	CD	denotes	1
T3809	2408-2409	,	denotes	,
T3810	2410-2411	-RRB-	denotes	]
T3811	2411-2412	.	denotes	.
T3812	2412-2588	sentence	denotes	Such a dietary management of the disease, however, is not entirely satisfactory especially in times of metabolic decompensation due to infection, injuries and other stressors.
T3813	2413-2417	PDT	denotes	Such
T3814	2428-2438	NN	denotes	management
T3815	2418-2419	DT	denotes	a
T3816	2420-2427	JJ	denotes	dietary
T3817	2464-2466	VBZ	denotes	is
T3818	2439-2441	IN	denotes	of
T3819	2442-2445	DT	denotes	the
T3820	2446-2453	NN	denotes	disease
T3821	2453-2455	,	denotes	,
T3822	2455-2462	RB	denotes	however
T3823	2462-2464	,	denotes	,
T3824	2467-2470	RB	denotes	not
T3825	2471-2479	RB	denotes	entirely
T3826	2480-2492	JJ	denotes	satisfactory
T3827	2493-2503	RB	denotes	especially
T3828	2504-2506	IN	denotes	in
T3829	2507-2512	NNS	denotes	times
T3830	2513-2515	IN	denotes	of
T3831	2516-2525	JJ	denotes	metabolic
T3832	2526-2540	NN	denotes	decompensation
T3833	2541-2544	IN	denotes	due
T3834	2545-2547	IN	denotes	to
T3835	2548-2557	NN	denotes	infection
T3836	2557-2559	,	denotes	,
T3837	2559-2567	NNS	denotes	injuries
T3838	2568-2571	CC	denotes	and
T3839	2572-2577	JJ	denotes	other
T3840	2578-2587	NNS	denotes	stressors
T3841	2587-2588	.	denotes	.
T3842	2588-2744	sentence	denotes	In spite of dietary intervention, there is significant mortality associated with MSUD and there is a high incidence of mental retardation in survivors [9].
T3843	2589-2591	IN	denotes	In
T3844	2629-2631	VBZ	denotes	is
T3845	2592-2597	NN	denotes	spite
T3846	2598-2600	IN	denotes	of
T3847	2601-2608	JJ	denotes	dietary
T3848	2609-2621	NN	denotes	intervention
T3849	2621-2623	,	denotes	,
T3850	2623-2628	EX	denotes	there
T3851	2632-2643	JJ	denotes	significant
T3852	2644-2653	NN	denotes	mortality
T3853	2654-2664	VBN	denotes	associated
T3854	2665-2669	IN	denotes	with
T3855	2670-2674	NN	denotes	MSUD
T3856	2675-2678	CC	denotes	and
T3857	2679-2684	EX	denotes	there
T3858	2685-2687	VBZ	denotes	is
T3859	2688-2689	DT	denotes	a
T3860	2695-2704	NN	denotes	incidence
T3861	2690-2694	JJ	denotes	high
T3862	2705-2707	IN	denotes	of
T3863	2708-2714	JJ	denotes	mental
T3864	2715-2726	NN	denotes	retardation
T3865	2727-2729	IN	denotes	in
T3866	2730-2739	NNS	denotes	survivors
T3867	2740-2741	-LRB-	denotes	[
T3868	2741-2742	CD	denotes	9
T3869	2742-2743	-RRB-	denotes	]
T3870	2743-2744	.	denotes	.
T3871	2744-2958	sentence	denotes	Because of the central role of the liver in amino acid metabolism and moderate/high levels of BCKDH activity in human liver [10-12], a few cases of MSUD have recently been treated by liver transplantation [13-16].
T3872	2745-2752	IN	denotes	Because
T3873	2917-2924	VBN	denotes	treated
T3874	2753-2755	IN	denotes	of
T3875	2756-2759	DT	denotes	the
T3876	2768-2772	NN	denotes	role
T3877	2760-2767	JJ	denotes	central
T3878	2773-2775	IN	denotes	of
T3879	2776-2779	DT	denotes	the
T3880	2780-2785	NN	denotes	liver
T3881	2786-2788	IN	denotes	in
T3882	2789-2794	NN	denotes	amino
T3883	2795-2799	NN	denotes	acid
T3884	2800-2810	NN	denotes	metabolism
T3885	2811-2814	CC	denotes	and
T3886	2815-2823	JJ	denotes	moderate
T3887	2824-2828	JJ	denotes	high
T3888	2823-2824	SYM	denotes	/
T3889	2829-2835	NNS	denotes	levels
T3890	2836-2838	IN	denotes	of
T3891	2839-2844	NN	denotes	BCKDH
T3892	2845-2853	NN	denotes	activity
T3893	2854-2856	IN	denotes	in
T3894	2857-2862	JJ	denotes	human
T3895	2863-2868	NN	denotes	liver
T3896	2869-2870	-LRB-	denotes	[
T3897	2870-2872	CD	denotes	10
T3898	2872-2873	SYM	denotes	-
T3899	2873-2875	CD	denotes	12
T3900	2875-2876	-RRB-	denotes	]
T3901	2876-2878	,	denotes	,
T3902	2878-2879	DT	denotes	a
T3903	2884-2889	NNS	denotes	cases
T3904	2880-2883	JJ	denotes	few
T3905	2890-2892	IN	denotes	of
T3906	2893-2897	NN	denotes	MSUD
T3907	2898-2902	VBP	denotes	have
T3908	2903-2911	RB	denotes	recently
T3909	2912-2916	VBN	denotes	been
T3910	2925-2927	IN	denotes	by
T3911	2928-2933	NN	denotes	liver
T3912	2934-2949	NN	denotes	transplantation
T3913	2950-2951	-LRB-	denotes	[
T3914	2951-2953	CD	denotes	13
T3915	2953-2954	SYM	denotes	-
T3916	2954-2956	CD	denotes	16
T3917	2956-2957	-RRB-	denotes	]
T3918	2957-2958	.	denotes	.
T3919	2958-3078	sentence	denotes	While the short-term outcome of liver transplantation is encouraging, long-term effects of this approach are not known.
T3920	2959-2964	IN	denotes	While
T3921	3013-3015	VBZ	denotes	is
T3922	2965-2968	DT	denotes	the
T3923	2980-2987	NN	denotes	outcome
T3924	2969-2974	JJ	denotes	short
T3925	2975-2979	NN	denotes	term
T3926	2974-2975	HYPH	denotes	-
T3927	2988-2990	IN	denotes	of
T3928	2991-2996	NN	denotes	liver
T3929	2997-3012	NN	denotes	transplantation
T3930	3072-3077	VBN	denotes	known
T3931	3016-3027	JJ	denotes	encouraging
T3932	3027-3029	,	denotes	,
T3933	3029-3033	JJ	denotes	long
T3934	3034-3038	NN	denotes	term
T3935	3033-3034	HYPH	denotes	-
T3936	3039-3046	NNS	denotes	effects
T3937	3047-3049	IN	denotes	of
T3938	3050-3054	DT	denotes	this
T3939	3055-3063	NN	denotes	approach
T3940	3064-3067	VBP	denotes	are
T3941	3068-3071	RB	denotes	not
T3942	3077-3078	.	denotes	.
T3943	3078-3217	sentence	denotes	However, these patients are now required to take immunosuppressant drugs for the rest of their lives, often with undesirable side effects.
T3944	3079-3086	RB	denotes	However
T3945	3111-3119	VBN	denotes	required
T3946	3086-3088	,	denotes	,
T3947	3088-3093	DT	denotes	these
T3948	3094-3102	NNS	denotes	patients
T3949	3103-3106	VBP	denotes	are
T3950	3107-3110	RB	denotes	now
T3951	3120-3122	TO	denotes	to
T3952	3123-3127	VB	denotes	take
T3953	3128-3145	NN	denotes	immunosuppressant
T3954	3146-3151	NNS	denotes	drugs
T3955	3152-3155	IN	denotes	for
T3956	3156-3159	DT	denotes	the
T3957	3160-3164	NN	denotes	rest
T3958	3165-3167	IN	denotes	of
T3959	3168-3173	PRP$	denotes	their
T3960	3174-3179	NNS	denotes	lives
T3961	3179-3181	,	denotes	,
T3962	3181-3186	RB	denotes	often
T3963	3187-3191	IN	denotes	with
T3964	3192-3203	JJ	denotes	undesirable
T3965	3209-3216	NNS	denotes	effects
T3966	3204-3208	NN	denotes	side
T3967	3216-3217	.	denotes	.
T3968	3217-3394	sentence	denotes	Moreover, the cost associated with liver transplantation and the availability of donor livers are additional limiting factors for the practicality of treatment of this disease.
T3969	3218-3226	RB	denotes	Moreover
T3970	3312-3315	VBP	denotes	are
T3971	3226-3228	,	denotes	,
T3972	3228-3231	DT	denotes	the
T3973	3232-3236	NN	denotes	cost
T3974	3237-3247	VBN	denotes	associated
T3975	3248-3252	IN	denotes	with
T3976	3253-3258	NN	denotes	liver
T3977	3259-3274	NN	denotes	transplantation
T3978	3275-3278	CC	denotes	and
T3979	3279-3282	DT	denotes	the
T3980	3283-3295	NN	denotes	availability
T3981	3296-3298	IN	denotes	of
T3982	3299-3304	NN	denotes	donor
T3983	3305-3311	NNS	denotes	livers
T3984	3316-3326	JJ	denotes	additional
T3985	3336-3343	NNS	denotes	factors
T3986	3327-3335	VBG	denotes	limiting
T3987	3344-3347	IN	denotes	for
T3988	3348-3351	DT	denotes	the
T3989	3352-3364	NN	denotes	practicality
T3990	3365-3367	IN	denotes	of
T3991	3368-3377	NN	denotes	treatment
T3992	3378-3380	IN	denotes	of
T3993	3381-3385	DT	denotes	this
T3994	3386-3393	NN	denotes	disease
T3995	3393-3394	.	denotes	.
T3996	3394-3530	sentence	denotes	Because of the current unsatisfactory options for the treatment of MSUD, there is a need for improved therapies to combat this disease.
T3997	3395-3402	IN	denotes	Because
T3998	3474-3476	VBZ	denotes	is
T3999	3403-3405	IN	denotes	of
T4000	3406-3409	DT	denotes	the
T4001	3433-3440	NNS	denotes	options
T4002	3410-3417	JJ	denotes	current
T4003	3418-3432	JJ	denotes	unsatisfactory
T4004	3441-3444	IN	denotes	for
T4005	3445-3448	DT	denotes	the
T4006	3449-3458	NN	denotes	treatment
T4007	3459-3461	IN	denotes	of
T4008	3462-3466	NN	denotes	MSUD
T4009	3466-3468	,	denotes	,
T4010	3468-3473	EX	denotes	there
T4011	3477-3478	DT	denotes	a
T4012	3479-3483	NN	denotes	need
T4013	3484-3487	IN	denotes	for
T4014	3488-3496	VBN	denotes	improved
T4015	3497-3506	NNS	denotes	therapies
T4016	3507-3509	TO	denotes	to
T4017	3510-3516	VB	denotes	combat
T4018	3517-3521	DT	denotes	this
T4019	3522-3529	NN	denotes	disease
T4020	3529-3530	.	denotes	.
T4021	3530-3669	sentence	denotes	An obstacle to developing novel treatments for MSUD has been the lack of a suitable animal model to perform necessary preclinical studies.
T4022	3531-3533	DT	denotes	An
T4023	3534-3542	NN	denotes	obstacle
T4024	3587-3591	VBN	denotes	been
T4025	3543-3545	IN	denotes	to
T4026	3546-3556	VBG	denotes	developing
T4027	3557-3562	JJ	denotes	novel
T4028	3563-3573	NNS	denotes	treatments
T4029	3574-3577	IN	denotes	for
T4030	3578-3582	NN	denotes	MSUD
T4031	3583-3586	VBZ	denotes	has
T4032	3592-3595	DT	denotes	the
T4033	3596-3600	NN	denotes	lack
T4034	3601-3603	IN	denotes	of
T4035	3604-3605	DT	denotes	a
T4036	3622-3627	NN	denotes	model
T4037	3606-3614	JJ	denotes	suitable
T4038	3615-3621	NN	denotes	animal
T4039	3628-3630	TO	denotes	to
T4040	3631-3638	VB	denotes	perform
T4041	3639-3648	JJ	denotes	necessary
T4042	3661-3668	NNS	denotes	studies
T4043	3649-3660	JJ	denotes	preclinical
T4044	3668-3669	.	denotes	.
T4045	3669-3824	sentence	denotes	Although a Hereford calf model with MSUD has been described [17-19], this model is neither readily available nor practical to perform preclinical studies.
T4046	3670-3678	IN	denotes	Although
T4047	3720-3729	VBN	denotes	described
T4048	3679-3680	DT	denotes	a
T4049	3695-3700	NN	denotes	model
T4050	3681-3689	NNP	denotes	Hereford
T4051	3690-3694	NN	denotes	calf
T4052	3701-3705	IN	denotes	with
T4053	3706-3710	NN	denotes	MSUD
T4054	3711-3714	VBZ	denotes	has
T4055	3715-3719	VBN	denotes	been
T4056	3750-3752	VBZ	denotes	is
T4057	3730-3731	-LRB-	denotes	[
T4058	3731-3733	CD	denotes	17
T4059	3733-3734	SYM	denotes	-
T4060	3734-3736	CD	denotes	19
T4061	3736-3737	-RRB-	denotes	]
T4062	3737-3739	,	denotes	,
T4063	3739-3743	DT	denotes	this
T4064	3744-3749	NN	denotes	model
T4065	3753-3760	CC	denotes	neither
T4066	3769-3778	JJ	denotes	available
T4067	3761-3768	RB	denotes	readily
T4068	3779-3782	CC	denotes	nor
T4069	3783-3792	JJ	denotes	practical
T4070	3793-3795	TO	denotes	to
T4071	3796-3803	VB	denotes	perform
T4072	3804-3815	JJ	denotes	preclinical
T4073	3816-3823	NNS	denotes	studies
T4074	3823-3824	.	denotes	.
T4075	3824-4014	sentence	denotes	Furthermore, comparison of this animal model with human MSUD has shown some differences in the pathology of the disease [1], making this animal a less desirable model for the human disease.
T4076	3825-3836	RB	denotes	Furthermore
T4077	3890-3895	VBN	denotes	shown
T4078	3836-3838	,	denotes	,
T4079	3838-3848	NN	denotes	comparison
T4080	3849-3851	IN	denotes	of
T4081	3852-3856	DT	denotes	this
T4082	3864-3869	NN	denotes	model
T4083	3857-3863	NN	denotes	animal
T4084	3870-3874	IN	denotes	with
T4085	3875-3880	JJ	denotes	human
T4086	3881-3885	NN	denotes	MSUD
T4087	3886-3889	VBZ	denotes	has
T4088	3896-3900	DT	denotes	some
T4089	3901-3912	NNS	denotes	differences
T4090	3913-3915	IN	denotes	in
T4091	3916-3919	DT	denotes	the
T4092	3920-3929	NN	denotes	pathology
T4093	3930-3932	IN	denotes	of
T4094	3933-3936	DT	denotes	the
T4095	3937-3944	NN	denotes	disease
T4096	3945-3946	-LRB-	denotes	[
T4097	3946-3947	CD	denotes	1
T4098	3947-3948	-RRB-	denotes	]
T4099	3948-3950	,	denotes	,
T4100	3950-3956	VBG	denotes	making
T4101	3957-3961	DT	denotes	this
T4102	3962-3968	NN	denotes	animal
T4103	3986-3991	NN	denotes	model
T4104	3969-3970	DT	denotes	a
T4105	3971-3975	RBR	denotes	less
T4106	3976-3985	JJ	denotes	desirable
T4107	3992-3995	IN	denotes	for
T4108	3996-3999	DT	denotes	the
T4109	4006-4013	NN	denotes	disease
T4110	4000-4005	JJ	denotes	human
T4111	4013-4014	.	denotes	.
T4112	4014-4141	sentence	denotes	Recently, a N-ethyl-N-nitrosourea (ENU)-induced mutant mouse that phenotypically resembles human MSUD has been described [20].
T4113	4015-4023	RB	denotes	Recently
T4114	4126-4135	VBN	denotes	described
T4115	4023-4025	,	denotes	,
T4116	4025-4026	DT	denotes	a
T4117	4037-4048	NN	denotes	nitrosourea
T4118	4027-4028	NN	denotes	N
T4119	4029-4034	NN	denotes	ethyl
T4120	4028-4029	HYPH	denotes	-
T4121	4034-4035	HYPH	denotes	-
T4122	4035-4036	NN	denotes	N
T4123	4036-4037	HYPH	denotes	-
T4124	4055-4062	VBN	denotes	induced
T4125	4049-4050	-LRB-	denotes	(
T4126	4050-4053	NN	denotes	ENU
T4127	4053-4054	-RRB-	denotes	)
T4128	4054-4055	HYPH	denotes	-
T4129	4070-4075	NN	denotes	mouse
T4130	4063-4069	NN	denotes	mutant
T4131	4076-4080	WDT	denotes	that
T4132	4096-4105	VBZ	denotes	resembles
T4133	4081-4095	RB	denotes	phenotypically
T4134	4106-4111	JJ	denotes	human
T4135	4112-4116	NN	denotes	MSUD
T4136	4117-4120	VBZ	denotes	has
T4137	4121-4125	VBN	denotes	been
T4138	4136-4137	-LRB-	denotes	[
T4139	4137-4139	CD	denotes	20
T4140	4139-4140	-RRB-	denotes	]
T4141	4140-4141	.	denotes	.
T4142	4141-4310	sentence	denotes	However, the mutation in this model disrupts a splice site in the mitochondrial branched-chain aminotransferase (BCAT) gene, not in BCKDH, the deficient enzyme in MSUD.
T4143	4142-4149	RB	denotes	However
T4144	4178-4186	VBZ	denotes	disrupts
T4145	4149-4151	,	denotes	,
T4146	4151-4154	DT	denotes	the
T4147	4155-4163	NN	denotes	mutation
T4148	4164-4166	IN	denotes	in
T4149	4167-4171	DT	denotes	this
T4150	4172-4177	NN	denotes	model
T4151	4187-4188	DT	denotes	a
T4152	4196-4200	NN	denotes	site
T4153	4189-4195	NN	denotes	splice
T4154	4201-4203	IN	denotes	in
T4155	4204-4207	DT	denotes	the
T4156	4237-4253	NN	denotes	aminotransferase
T4157	4208-4221	JJ	denotes	mitochondrial
T4158	4222-4230	VBN	denotes	branched
T4159	4231-4236	NN	denotes	chain
T4160	4230-4231	HYPH	denotes	-
T4161	4261-4265	NN	denotes	gene
T4162	4254-4255	-LRB-	denotes	(
T4163	4255-4259	NN	denotes	BCAT
T4164	4259-4260	-RRB-	denotes	)
T4165	4265-4267	,	denotes	,
T4166	4267-4270	RB	denotes	not
T4167	4271-4273	IN	denotes	in
T4168	4274-4279	NN	denotes	BCKDH
T4169	4279-4281	,	denotes	,
T4170	4281-4284	DT	denotes	the
T4171	4295-4301	NN	denotes	enzyme
T4172	4285-4294	JJ	denotes	deficient
T4173	4302-4304	IN	denotes	in
T4174	4305-4309	NN	denotes	MSUD
T4175	4309-4310	.	denotes	.
T4176	4310-4428	sentence	denotes	Because the mutation is not in BCKDH, the validity of this mutant mouse line for modeling human MSUD is questionable.
T4177	4311-4318	IN	denotes	Because
T4178	4332-4334	VBZ	denotes	is
T4179	4319-4322	DT	denotes	the
T4180	4323-4331	NN	denotes	mutation
T4181	4412-4414	VBZ	denotes	is
T4182	4335-4338	RB	denotes	not
T4183	4339-4341	IN	denotes	in
T4184	4342-4347	NN	denotes	BCKDH
T4185	4347-4349	,	denotes	,
T4186	4349-4352	DT	denotes	the
T4187	4353-4361	NN	denotes	validity
T4188	4362-4364	IN	denotes	of
T4189	4365-4369	DT	denotes	this
T4190	4383-4387	NN	denotes	line
T4191	4370-4376	NN	denotes	mutant
T4192	4377-4382	NN	denotes	mouse
T4193	4388-4391	IN	denotes	for
T4194	4392-4400	VBG	denotes	modeling
T4195	4407-4411	NN	denotes	MSUD
T4196	4401-4406	JJ	denotes	human
T4197	4415-4427	JJ	denotes	questionable
T4198	4427-4428	.	denotes	.
T4199	4428-4615	sentence	denotes	The objective of the present study was to create genetically engineered murine models of MSUD that mimic the pathology of the classic and intermediate variant forms of the human disease.
T4200	4429-4432	DT	denotes	The
T4201	4433-4442	NN	denotes	objective
T4202	4464-4467	VBD	denotes	was
T4203	4443-4445	IN	denotes	of
T4204	4446-4449	DT	denotes	the
T4205	4458-4463	NN	denotes	study
T4206	4450-4457	JJ	denotes	present
T4207	4468-4470	TO	denotes	to
T4208	4471-4477	VB	denotes	create
T4209	4478-4489	RB	denotes	genetically
T4210	4490-4500	VBN	denotes	engineered
T4211	4508-4514	NNS	denotes	models
T4212	4501-4507	JJ	denotes	murine
T4213	4515-4517	IN	denotes	of
T4214	4518-4522	NN	denotes	MSUD
T4215	4523-4527	WDT	denotes	that
T4216	4528-4533	VBP	denotes	mimic
T4217	4534-4537	DT	denotes	the
T4218	4538-4547	NN	denotes	pathology
T4219	4548-4550	IN	denotes	of
T4220	4551-4554	DT	denotes	the
T4221	4588-4593	NNS	denotes	forms
T4222	4555-4562	JJ	denotes	classic
T4223	4563-4566	CC	denotes	and
T4224	4567-4579	JJ	denotes	intermediate
T4225	4580-4587	JJ	denotes	variant
T4226	4594-4596	IN	denotes	of
T4227	4597-4600	DT	denotes	the
T4228	4607-4614	NN	denotes	disease
T4229	4601-4606	JJ	denotes	human
T4230	4614-4615	.	denotes	.
T4231	4615-4755	sentence	denotes	The classic model was created by targeted inactivation of the E2 subunit of BCKDH by homologous recombination in embryonic stem (ES) cells.
T4232	4616-4619	DT	denotes	The
T4233	4628-4633	NN	denotes	model
T4234	4620-4627	JJ	denotes	classic
T4235	4638-4645	VBN	denotes	created
T4236	4634-4637	VBD	denotes	was
T4237	4646-4648	IN	denotes	by
T4238	4649-4657	VBN	denotes	targeted
T4239	4658-4670	NN	denotes	inactivation
T4240	4671-4673	IN	denotes	of
T4241	4674-4677	DT	denotes	the
T4242	4681-4688	NN	denotes	subunit
T4243	4678-4680	NN	denotes	E2
T4244	4689-4691	IN	denotes	of
T4245	4692-4697	NN	denotes	BCKDH
T4246	4698-4700	IN	denotes	by
T4247	4701-4711	JJ	denotes	homologous
T4248	4712-4725	NN	denotes	recombination
T4249	4726-4728	IN	denotes	in
T4250	4729-4738	JJ	denotes	embryonic
T4251	4745-4747	NN	denotes	ES
T4252	4739-4743	NN	denotes	stem
T4253	4744-4745	-LRB-	denotes	(
T4254	4749-4754	NNS	denotes	cells
T4255	4747-4748	-RRB-	denotes	)
T4256	4754-4755	.	denotes	.
T4257	4755-4852	sentence	denotes	The model of intermediate MSUD was created by partial transgenic rescue of the E2 gene knockout.
T4258	4756-4759	DT	denotes	The
T4259	4760-4765	NN	denotes	model
T4260	4791-4798	VBN	denotes	created
T4261	4766-4768	IN	denotes	of
T4262	4769-4781	JJ	denotes	intermediate
T4263	4782-4786	NN	denotes	MSUD
T4264	4787-4790	VBD	denotes	was
T4265	4799-4801	IN	denotes	by
T4266	4802-4809	JJ	denotes	partial
T4267	4821-4827	NN	denotes	rescue
T4268	4810-4820	JJ	denotes	transgenic
T4269	4828-4830	IN	denotes	of
T4270	4831-4834	DT	denotes	the
T4271	4843-4851	NN	denotes	knockout
T4272	4835-4837	NN	denotes	E2
T4273	4838-4842	NN	denotes	gene
T4274	4851-4852	.	denotes	.
T4275	4852-4942	sentence	denotes	This report describes the generation and characterization of these murine models of MSUD.
T4276	4853-4857	DT	denotes	This
T4277	4858-4864	NN	denotes	report
T4278	4865-4874	VBZ	denotes	describes
T4279	4875-4878	DT	denotes	the
T4280	4879-4889	NN	denotes	generation
T4281	4890-4893	CC	denotes	and
T4282	4894-4910	NN	denotes	characterization
T4283	4911-4913	IN	denotes	of
T4284	4914-4919	DT	denotes	these
T4285	4927-4933	NNS	denotes	models
T4286	4920-4926	JJ	denotes	murine
T4287	4934-4936	IN	denotes	of
T4288	4937-4941	NN	denotes	MSUD
T4289	4941-4942	.	denotes	.
T4290	4942-5079	sentence	denotes	These models will allow for the development of novel treatment approaches, such as gene or stem cell therapies, to ultimately cure MSUD.
T4291	4943-4948	DT	denotes	These
T4292	4949-4955	NNS	denotes	models
T4293	4961-4966	VB	denotes	allow
T4294	4956-4960	MD	denotes	will
T4295	4967-4970	IN	denotes	for
T4296	4971-4974	DT	denotes	the
T4297	4975-4986	NN	denotes	development
T4298	4987-4989	IN	denotes	of
T4299	4990-4995	JJ	denotes	novel
T4300	5006-5016	NNS	denotes	approaches
T4301	4996-5005	NN	denotes	treatment
T4302	5016-5018	,	denotes	,
T4303	5018-5022	JJ	denotes	such
T4304	5023-5025	IN	denotes	as
T4305	5026-5030	NN	denotes	gene
T4306	5044-5053	NNS	denotes	therapies
T4307	5031-5033	CC	denotes	or
T4308	5034-5038	NN	denotes	stem
T4309	5039-5043	NN	denotes	cell
T4310	5053-5055	,	denotes	,
T4311	5055-5057	TO	denotes	to
T4312	5069-5073	VB	denotes	cure
T4313	5058-5068	RB	denotes	ultimately
T4314	5074-5078	NN	denotes	MSUD
T4315	5078-5079	.	denotes	.
T3304	11-16	NN	denotes	Maple
T3305	17-22	NN	denotes	Syrup
T3306	29-36	NN	denotes	Disease
T3307	23-28	NN	denotes	Urine
T3308	44-46	VBZ	denotes	is
T3309	37-38	-LRB-	denotes	(
T3310	38-42	NN	denotes	MSUD
T3311	42-43	-RRB-	denotes	)
T3312	47-48	DT	denotes	a
T3313	57-65	NN	denotes	disorder
T3314	49-56	JJ	denotes	genetic
T3315	66-72	VBN	denotes	caused
T3316	73-75	IN	denotes	by
T3317	76-77	DT	denotes	a
R348	T3304	T3305	compound	Maple,Syrup
R349	T3305	T3306	compound	Syrup,Disease
R350	T3306	T3308	nsubj	Disease,is
R351	T3307	T3306	compound	Urine,Disease
R352	T3309	T3306	punct	(,Disease
R353	T3310	T3306	appos	MSUD,Disease
R354	T3311	T3308	punct	),is
R355	T3312	T3313	det	a,disorder
R356	T3313	T3308	attr	disorder,is
R357	T3314	T3313	amod	genetic,disorder
R358	T3315	T3313	acl	caused,disorder
R359	T3316	T3315	agent	by,caused
R360	T3317	T3318	det	a,deficiency
R361	T3318	T3316	pobj	deficiency,by
R362	T3319	T3318	prep	of,deficiency
R363	T3320	T3321	amod	branched,chain
R364	T3321	T3323	compound	chain,dehydrogenase
R365	T3322	T3321	punct	-,chain
R366	T3323	T3319	pobj	dehydrogenase,of
R367	T3324	T3325	compound	keto,acid
R368	T3325	T3323	compound	acid,dehydrogenase
R369	T3326	T3323	punct	(,dehydrogenase
R370	T3327	T3323	appos	BCKDH,dehydrogenase
R371	T3328	T3318	punct	),deficiency
R372	T3329	T3318	punct	", ",deficiency
R373	T3330	T3331	det	a,complex
R374	T3331	T3318	appos	complex,deficiency
R375	T3332	T3331	amod	mitochondrial,complex
R376	T3333	T3331	compound	multienzyme,complex
R377	T3334	T3331	amod	responsible,complex
R378	T3335	T3334	prep	for,responsible
R379	T3336	T3337	det	the,decarboxylation
R380	T3337	T3335	pobj	decarboxylation,for
R381	T3338	T3337	amod	oxidative,decarboxylation
R382	T3339	T3337	prep	of,decarboxylation
R383	T3423	T3422	prep	of,%
R384	T3340	T3341	amod	branched,chain
R385	T3424	T3423	pobj	normal,of
R386	T3425	T3426	punct	[,1
R387	T3341	T3343	compound	chain,acids
R388	T3426	T3402	parataxis	1,have
R389	T3427	T3426	punct	],1
R390	T3428	T3402	punct	.,have
R391	T3430	T3431	det	These,patients
R392	T3431	T3432	nsubj	patients,show
R393	T3433	T3434	advmod	markedly,elevated
R394	T3434	T3435	amod	elevated,levels
R395	T3435	T3432	dobj	levels,show
R396	T3342	T3341	punct	-,chain
R397	T3436	T3435	prep	of,levels
R398	T3437	T3436	pobj	BCAA,of
R399	T3438	T3432	prep	in,show
R400	T3439	T3438	pobj	blood,in
R401	T3343	T3339	pobj	acids,of
R402	T3440	T3439	cc	and,blood
R403	T3441	T3442	amod	other,fluids
R404	T3344	T3343	compound	keto,acids
R405	T3442	T3439	conj	fluids,blood
R406	T3443	T3442	compound	body,fluids
R407	T3444	T3445	punct	[,1
R408	T3345	T3343	acl	derived,acids
R409	T3445	T3432	parataxis	1,show
R410	T3446	T3445	punct	],1
R411	T3447	T3432	punct	.,show
R412	T3346	T3345	prep	from,derived
R413	T3449	T3450	prep	Besides,are
R414	T3347	T3348	amod	branched,chain
R415	T3451	T3452	det	the,form
R416	T3452	T3449	pobj	form,Besides
R417	T3348	T3350	compound	chain,acids
R418	T3453	T3452	amod	classic,form
R419	T3454	T3450	punct	", ",are
R420	T3455	T3450	expl	there,are
R421	T3349	T3348	punct	-,chain
R422	T3456	T3457	amod	other,variants
R423	T3350	T3346	pobj	acids,from
R424	T3457	T3450	attr	variants,are
R425	T3458	T3457	prep	of,variants
R426	T3459	T3460	det	the,disease
R427	T3460	T3458	pobj	disease,of
R428	T3351	T3350	compound	amino,acids
R429	T3461	T3450	punct	.,are
R430	T3463	T3464	nsubj	Patients,show
R431	T3352	T3350	punct	(,acids
R432	T3465	T3463	prep	with,Patients
R433	T3466	T3467	det	the,form
R434	T3353	T3350	appos	BCAA,acids
R435	T3354	T3350	punct	),acids
R436	T3467	T3465	pobj	form,with
R437	T3468	T3467	amod	intermediate,form
R438	T3355	T3350	punct	", ",acids
R439	T3469	T3467	prep	of,form
R440	T3470	T3471	det	the,disease
R441	T3471	T3469	pobj	disease,of
R442	T3472	T3473	compound	BCKDH,activity
R443	T3473	T3464	dobj	activity,show
R444	T3474	T3473	prep	in,activity
R445	T3356	T3350	conj	leucine,acids
R446	T3475	T3476	det	the,range
R447	T3476	T3474	pobj	range,in
R448	T3477	T3476	prep	of,range
R449	T3357	T3356	punct	", ",leucine
R450	T3478	T3479	quantmod	3,30
R451	T3479	T3481	nummod	30,%
R452	T3480	T3479	punct	–,30
R453	T3358	T3356	conj	isoleucine,leucine
R454	T3481	T3477	pobj	%,of
R455	T3482	T3481	prep	of,%
R456	T3483	T3482	pobj	normal,of
R457	T3359	T3358	cc	and,isoleucine
R458	T3484	T3464	punct	.,show
R459	T3486	T3487	prep	In,delayed
R460	T3360	T3358	conj	valine,isoleucine
R461	T3488	T3489	amod	such,patients
R462	T3489	T3486	pobj	patients,In
R463	T3361	T3362	punct	(,see
R464	T3490	T3491	det	the,onset
R465	T3491	T3487	nsubjpass	onset,delayed
R466	T3362	T3308	parataxis	see,is
R467	T3492	T3491	prep	of,onset
R468	T3493	T3494	det	the,disease
R469	T3494	T3492	pobj	disease,of
R470	T3495	T3487	auxpass	is,delayed
R471	T3363	T3362	prep	for,see
R472	T3496	T3487	punct	", ",delayed
R473	T3497	T3487	cc	but,delayed
R474	T3498	T3499	expl	there,are
R475	T3364	T3363	pobj	review,for
R476	T3499	T3487	conj	are,delayed
R477	T3365	T3362	punct	", ",see
R478	T3500	T3501	advmod	persistently,elevated
R479	T3501	T3502	amod	elevated,levels
R480	T3502	T3499	attr	levels,are
R481	T3503	T3502	prep	of,levels
R482	T3366	T3362	punct	: ,see
R483	T3504	T3503	pobj	BCAA,of
R484	T3505	T3506	punct	[,1
R485	T3367	T3362	punct	[,see
R486	T3506	T3499	parataxis	1,are
R487	T3507	T3506	punct	],1
R488	T3508	T3499	punct	.,are
R489	T3368	T3362	dobj	1,see
R490	T3510	T3511	nsubj	Patients,show
R491	T3369	T3362	punct	],see
R492	T3512	T3510	prep	with,Patients
R493	T3513	T3514	det	the,form
R494	T3514	T3512	pobj	form,with
R495	T3515	T3514	amod	intermittent,form
R496	T3516	T3514	prep	of,form
R497	T3370	T3362	punct	),see
R498	T3517	T3516	pobj	MSUD,of
R499	T3518	T3519	compound	BCKDH,activity
R500	T3519	T3511	dobj	activity,show
R501	T3520	T3511	prep	in,show
R502	T3371	T3308	punct	.,is
R503	T3521	T3522	det	the,range
R504	T3522	T3520	pobj	range,in
R505	T3523	T3522	prep	of,range
R506	T3373	T3374	nsubj	Patients,show
R507	T3524	T3525	quantmod	5,20
R508	T3525	T3527	nummod	20,%
R509	T3526	T3525	punct	–,20
R510	T3527	T3523	pobj	%,of
R511	T3375	T3373	prep	with,Patients
R512	T3528	T3511	cc	and,show
R513	T3376	T3375	pobj	MSUD,with
R514	T3377	T3374	punct	", ",show
R515	T3378	T3374	prep	depending,show
R516	T3529	T3530	prep	during,are
R517	T3530	T3511	conj	are,show
R518	T3379	T3378	prep	on,depending
R519	T3531	T3532	det	the,phase
R520	T3532	T3529	pobj	phase,during
R521	T3380	T3381	det	the,mutation
R522	T3533	T3532	amod	asymptomatic,phase
R523	T3534	T3535	det	the,levels
R524	T3535	T3530	nsubj	levels,are
R525	T3381	T3379	pobj	mutation,on
R526	T3536	T3535	compound	blood,levels
R527	T3537	T3535	compound	BCAA,levels
R528	T3538	T3530	acomp	normal,are
R529	T3382	T3374	punct	", ",show
R530	T3539	T3540	punct	[,1
R531	T3540	T3530	parataxis	1,are
R532	T3383	T3384	amod	variable,degrees
R533	T3541	T3540	punct	],1
R534	T3542	T3530	punct	.,are
R535	T3384	T3374	dobj	degrees,show
R536	T3544	T3545	det	The,incidence
R537	T3545	T3547	nsubj	incidence,is
R538	T3546	T3545	amod	overall,incidence
R539	T3385	T3384	prep	of,degrees
R540	T3548	T3545	prep	of,incidence
R541	T3386	T3387	compound	enzyme,deficiency
R542	T3549	T3548	pobj	MSUD,of
R543	T3550	T3545	prep	in,incidence
R544	T3551	T3552	det	the,population
R545	T3552	T3550	pobj	population,in
R546	T3553	T3552	amod	general,population
R547	T3387	T3385	pobj	deficiency,of
R548	T3554	T3555	nummod	1,"185,000"
R549	T3555	T3547	attr	"185,000",is
R550	T3556	T3555	punct	:,"185,000"
R551	T3388	T3387	acl	leading,deficiency
R552	T3557	T3558	punct	[,1
R553	T3558	T3547	parataxis	1,is
R554	T3559	T3558	punct	],1
R555	T3389	T3388	prep	to,leading
R556	T3560	T3547	punct	", ",is
R557	T3561	T3547	cc	and,is
R558	T3562	T3563	prep	in,estimated
R559	T3390	T3391	amod	several,phenotypes
R560	T3563	T3547	conj	estimated,is
R561	T3391	T3389	pobj	phenotypes,to
R562	T3564	T3565	amod	certain,groups
R563	T3565	T3562	pobj	groups,in
R564	T3566	T3565	compound	population,groups
R565	T3392	T3391	amod	different,phenotypes
R566	T3567	T3565	punct	", ",groups
R567	T3568	T3569	amod	such,as
R568	T3569	T3565	prep	as,groups
R569	T3570	T3569	pobj	Mennonites,as
R570	T3393	T3391	amod	clinical,phenotypes
R571	T3571	T3570	prep	of,Mennonites
R572	T3572	T3571	pobj	Pennsylvania,of
R573	T3394	T3395	punct	[,1
R574	T3573	T3563	punct	", ",estimated
R575	T3574	T3575	det	the,incidence
R576	T3575	T3563	nsubjpass	incidence,estimated
R577	T3576	T3563	auxpass	is,estimated
R578	T3395	T3374	parataxis	1,show
R579	T3577	T3578	aux	to,be
R580	T3578	T3563	xcomp	be,estimated
R581	T3396	T3395	punct	],1
R582	T3579	T3578	prep	as,be
R583	T3580	T3579	amod	high,as
R584	T3581	T3580	prep	as,high
R585	T3397	T3374	punct	.,show
R586	T3582	T3583	nummod	1,176
R587	T3583	T3581	pobj	176,as
R588	T3584	T3583	punct	:,176
R589	T3399	T3400	advmod	Approximately,75
R590	T3585	T3586	punct	[,2
R591	T3586	T3563	parataxis	2,estimated
R592	T3587	T3586	punct	],2
R593	T3588	T3563	punct	.,estimated
R594	T3400	T3401	nummod	75,%
R595	T3590	T3591	det	The,complex
R596	T3401	T3402	nsubj	%,have
R597	T3591	T3593	nsubj	complex,consists
R598	T3592	T3591	compound	BCKDH,complex
R599	T3594	T3591	punct	", ",complex
R600	T3595	T3596	det	the,enzyme
R601	T3596	T3591	appos	enzyme,complex
R602	T3403	T3401	prep	of,%
R603	T3597	T3596	amod	deficient,enzyme
R604	T3598	T3596	prep	in,enzyme
R605	T3599	T3598	pobj	MSUD,in
R606	T3600	T3593	punct	", ",consists
R607	T3601	T3593	prep	of,consists
R608	T3404	T3405	compound	MSUD,patients
R609	T3602	T3603	nummod	three,proteins
R610	T3603	T3601	pobj	proteins,of
R611	T3604	T3603	amod	catalytic,proteins
R612	T3405	T3403	pobj	patients,of
R613	T3605	T3603	punct	", ",proteins
R614	T3606	T3607	det	a,decarboxylase
R615	T3607	T3603	appos	decarboxylase,proteins
R616	T3406	T3407	det	the,form
R617	T3608	T3609	punct	(,E1
R618	T3609	T3607	parataxis	E1,decarboxylase
R619	T3610	T3609	punct	),E1
R620	T3407	T3402	dobj	form,have
R621	T3611	T3607	punct	", ",decarboxylase
R622	T3612	T3613	det	a,transacylase
R623	T3408	T3407	amod	classic,form
R624	T3613	T3607	conj	transacylase,decarboxylase
R625	T3614	T3613	compound	dihydrolipoyl,transacylase
R626	T3615	T3616	punct	(,E2
R627	T3409	T3407	prep	of,form
R628	T3616	T3613	parataxis	E2,transacylase
R629	T3617	T3616	punct	),E2
R630	T3618	T3613	punct	", ",transacylase
R631	T3410	T3411	det	the,disease
R632	T3619	T3613	cc	and,transacylase
R633	T3620	T3621	det	a,dehydrogenase
R634	T3621	T3613	conj	dehydrogenase,transacylase
R635	T3622	T3621	compound	dihydrolipoyl,dehydrogenase
R636	T3411	T3409	pobj	disease,of
R637	T3623	T3624	punct	(,E3
R638	T3624	T3621	parataxis	E3,dehydrogenase
R639	T3625	T3624	punct	),E3
R640	T3412	T3407	prep	with,form
R641	T3626	T3593	punct	.,consists
R642	T3413	T3414	compound	BCKDH,activity
R644	T3629	T3631	nsubj	component,is
R645	T3630	T3629	compound	E1,component
R646	T3414	T3412	pobj	activity,with
R647	T3632	T3633	det	a,heterotetramer
R648	T3633	T3631	attr	heterotetramer,is
R649	T3415	T3414	prep	in,activity
R650	T3416	T3417	det	the,range
R651	T3634	T3633	acl	composed,heterotetramer
R652	T3417	T3415	pobj	range,in
R653	T3635	T3634	prep	of,composed
R654	T3636	T3637	nummod	two,α
R655	T3637	T3638	nmod	α,subunits
R656	T3418	T3417	prep	of,range
R657	T3638	T3635	pobj	subunits,of
R658	T3639	T3637	cc	and,α
R659	T3419	T3420	quantmod	0,2
R660	T3640	T3641	nummod	two,β
R661	T3641	T3637	conj	β,α
R662	T3642	T3643	punct	[,3
R663	T3420	T3422	nummod	2,%
R664	T3643	T3631	parataxis	3,is
R665	T3644	T3643	punct	],3
R666	T3645	T3631	punct	.,is
R667	T3421	T3420	punct	–,2
R668	T3647	T3648	det	The,components
R669	T3422	T3418	pobj	%,of
R670	T3648	T3652	nsubj	components,are
R671	T3649	T3648	nmod	E1,components
R672	T3650	T3649	cc	and,E1
R673	T3739	T3737	conj	E2,E1
R674	T3651	T3649	conj	E2,E1
R675	T3653	T3652	acomp	specific,are
R676	T3654	T3653	prep	to,specific
R677	T3655	T3654	pobj	BCKDH,to
R678	T3740	T3736	prep	of,subunits
R679	T3656	T3652	punct	", ",are
R680	T3657	T3658	mark	whereas,used
R681	T3658	T3652	advcl	used,are
R682	T3741	T3740	pobj	BCKDH,of
R683	T3659	T3658	nsubjpass	E3,used
R684	T3660	T3658	auxpass	is,used
R685	T3661	T3658	advmod	also,used
R686	T3742	T3730	aux	have,described
R687	T3662	T3658	agent	by,used
R688	T3663	T3664	nmod	pyruvate,complexes
R689	T3664	T3662	pobj	complexes,by
R690	T3743	T3730	auxpass	been,described
R691	T3665	T3663	cc	and,pyruvate
R692	T3666	T3667	compound	α,ketoglutarate
R693	T3745	T3744	punct	", ",are
R694	T3667	T3663	conj	ketoglutarate,pyruvate
R695	T3668	T3667	punct	-,ketoglutarate
R696	T3669	T3664	compound	dehydrogenase,complexes
R697	T3670	T3671	punct	[,3
R698	T3671	T3664	parataxis	3,complexes
R699	T3746	T3744	advmod	however,are
R700	T3672	T3671	punct	],3
R701	T3673	T3664	cc	and,complexes
R702	T3674	T3675	det	the,system
R703	T3675	T3664	conj	system,complexes
R704	T3676	T3677	compound	glycine,cleavage
R705	T3677	T3675	compound	cleavage,system
R706	T3678	T3679	punct	[,4
R707	T3747	T3744	punct	", ",are
R708	T3679	T3675	parataxis	4,system
R709	T3680	T3679	punct	],4
R710	T3681	T3652	punct	.,are
R711	T3748	T3749	det	the,majority
R712	T3683	T3684	nsubjpass	BCKDH,associated
R713	T3749	T3744	nsubj	majority,are
R714	T3685	T3684	auxpass	is,associated
R715	T3686	T3684	advmod	also,associated
R716	T3750	T3749	prep	of,majority
R717	T3687	T3684	prep	with,associated
R718	T3688	T3689	nummod	two,proteins
R719	T3689	T3687	pobj	proteins,with
R720	T3751	T3752	compound	MSUD,mutations
R721	T3690	T3689	amod	regulatory,proteins
R722	T3691	T3689	punct	", ",proteins
R723	T3692	T3693	det	a,kinase
R724	T3693	T3689	appos	kinase,proteins
R725	T3752	T3750	pobj	mutations,of
R726	T3694	T3693	amod	specific,kinase
R727	T3695	T3693	cc	and,kinase
R728	T3753	T3752	acl	identified,mutations
R729	T3696	T3697	det	a,phosphatase
R730	T3697	T3693	conj	phosphatase,kinase
R731	T3698	T3699	dep	which,regulate
R732	T3699	T3689	relcl	regulate,proteins
R733	T3754	T3755	advmod	thus,far
R734	T3700	T3701	det	the,activity
R735	T3701	T3699	dobj	activity,regulate
R736	T3702	T3701	prep	of,activity
R737	T3755	T3753	advmod	far,identified
R738	T3703	T3704	det	this,enzyme
R739	T3704	T3702	pobj	enzyme,of
R740	T3705	T3699	prep	through,regulate
R741	T3756	T3744	prep	in,are
R742	T3706	T3707	det	a,cycle
R743	T3707	T3705	pobj	cycle,through
R744	T3757	T3758	det	the,subunit
R745	T3708	T3707	nmod	phosphorylation,cycle
R746	T3709	T3710	punct	(,inactivation
R747	T3710	T3708	parataxis	inactivation,phosphorylation
R748	T3758	T3756	pobj	subunit,in
R749	T3711	T3710	punct	),inactivation
R750	T3712	T3708	cc	and,phosphorylation
R751	T3713	T3708	conj	dephosphorylation,phosphorylation
R752	T3759	T3758	compound	E2,subunit
R753	T3714	T3715	punct	(,activation
R754	T3715	T3713	parataxis	activation,dephosphorylation
R755	T3716	T3715	punct	),activation
R756	T3717	T3707	prep	of,cycle
R757	T3718	T3719	det	the,subunit
R758	T3719	T3717	pobj	subunit,of
R759	T3760	T3761	punct	[,7
R760	T3720	T3719	compound	E1,subunit
R761	T3721	T3719	compound	α,subunit
R762	T3722	T3723	punct	[,6
R763	T3761	T3744	parataxis	7,are
R764	T3723	T3684	parataxis	6,associated
R765	T3762	T3761	nummod	1,7
R766	T3724	T3723	nummod	5,6
R767	T3725	T3723	punct	",",6
R768	T3726	T3723	punct	],6
R769	T3763	T3761	punct	",",7
R770	T3727	T3684	punct	.,associated
R771	T3764	T3761	punct	],7
R772	T3765	T3744	punct	.,are
R773	T3767	T3768	prep	To,associated
R774	T3729	T3730	nsubjpass	Mutations,described
R775	T3730	T3744	ccomp	described,are
R776	T3731	T3729	prep	in,Mutations
R777	T3769	T3767	pobj	date,To
R778	T3732	T3733	det	the,genes
R779	T3733	T3731	pobj	genes,in
R780	T3734	T3733	prep	of,genes
R781	T3735	T3736	det	the,subunits
R782	T3770	T3768	punct	", ",associated
R783	T3736	T3734	pobj	subunits,of
R784	T3737	T3736	nmod	E1,subunits
R785	T3738	T3737	cc	and,E1
R786	T3771	T3768	nsubjpass	cases,associated
R787	T3772	T3771	prep	of,cases
R788	T3773	T3772	pobj	MSUD,of
R789	T3845	T3843	pobj	spite,In
R790	T3774	T3768	aux	have,associated
R791	T3846	T3845	prep	of,spite
R792	T3847	T3848	amod	dietary,intervention
R793	T3775	T3768	neg	not,associated
R794	T3848	T3846	pobj	intervention,of
R795	T3849	T3844	punct	", ",is
R796	T3850	T3844	expl	there,is
R797	T3776	T3768	auxpass	been,associated
R798	T3851	T3852	amod	significant,mortality
R799	T3852	T3844	attr	mortality,is
R800	T3777	T3768	prep	with,associated
R801	T3853	T3852	acl	associated,mortality
R802	T3854	T3853	prep	with,associated
R803	T3778	T3777	pobj	defects,with
R804	T3855	T3854	pobj	MSUD,with
R805	T3856	T3844	cc	and,is
R806	T3857	T3858	expl	there,is
R807	T3858	T3844	conj	is,is
R808	T3859	T3860	det	a,incidence
R809	T3860	T3858	attr	incidence,is
R810	T3779	T3778	prep	in,defects
R811	T3861	T3860	amod	high,incidence
R812	T3862	T3860	prep	of,incidence
R813	T3863	T3864	amod	mental,retardation
R814	T3864	T3862	pobj	retardation,of
R815	T3780	T3781	det	the,kinase
R816	T3865	T3858	prep	in,is
R817	T3866	T3865	pobj	survivors,in
R818	T3867	T3868	punct	[,9
R819	T3781	T3779	pobj	kinase,in
R820	T3868	T3858	parataxis	9,is
R821	T3869	T3868	punct	],9
R822	T3870	T3844	punct	.,is
R823	T3782	T3781	amod	regulatory,kinase
R824	T3872	T3873	prep	Because,treated
R825	T3783	T3781	cc	and,kinase
R826	T3874	T3872	pcomp	of,Because
R827	T3875	T3876	det	the,role
R828	T3784	T3781	conj	phosphatase,kinase
R829	T3876	T3872	pobj	role,Because
R830	T3877	T3876	amod	central,role
R831	T3878	T3876	prep	of,role
R832	T3785	T3786	punct	[,1
R833	T3879	T3880	det	the,liver
R834	T3880	T3878	pobj	liver,of
R835	T3881	T3876	prep	in,role
R836	T3786	T3768	parataxis	1,associated
R837	T3882	T3883	compound	amino,acid
R838	T3883	T3884	compound	acid,metabolism
R839	T3884	T3881	pobj	metabolism,in
R840	T3787	T3786	punct	],1
R841	T3885	T3876	cc	and,role
R842	T3886	T3887	amod	moderate,high
R843	T3887	T3889	amod	high,levels
R844	T3788	T3768	punct	.,associated
R845	T3888	T3887	punct	/,high
R846	T3889	T3876	conj	levels,role
R847	T3890	T3889	prep	of,levels
R848	T3790	T3791	amod	Current,management
R849	T3891	T3892	compound	BCKDH,activity
R850	T3892	T3890	pobj	activity,of
R851	T3893	T3892	prep	in,activity
R852	T3894	T3895	amod	human,liver
R853	T3791	T3792	nsubj	management,relies
R854	T3895	T3893	pobj	liver,in
R855	T3793	T3791	prep	of,management
R856	T3896	T3897	punct	[,10
R857	T3794	T3795	compound	MSUD,patients
R858	T3897	T3889	parataxis	10,levels
R859	T3898	T3899	punct	-,12
R860	T3899	T3897	prep	12,10
R861	T3900	T3897	punct	],10
R862	T3901	T3873	punct	", ",treated
R863	T3902	T3903	det	a,cases
R864	T3795	T3793	pobj	patients,of
R865	T3903	T3873	nsubjpass	cases,treated
R866	T3904	T3903	amod	few,cases
R867	T3796	T3792	prep	on,relies
R868	T3905	T3903	prep	of,cases
R869	T3906	T3905	pobj	MSUD,of
R870	T3907	T3873	aux	have,treated
R871	T3797	T3798	det	a,restriction
R872	T3908	T3873	advmod	recently,treated
R873	T3909	T3873	auxpass	been,treated
R874	T3910	T3873	prep	by,treated
R875	T3798	T3796	pobj	restriction,on
R876	T3911	T3912	compound	liver,transplantation
R877	T3799	T3798	amod	strict,restriction
R878	T3912	T3910	pobj	transplantation,by
R879	T3800	T3798	amod	lifelong,restriction
R880	T3913	T3914	punct	[,13
R881	T3801	T3798	amod	dietary,restriction
R882	T3914	T3873	parataxis	13,treated
R883	T3915	T3916	punct	-,16
R884	T3916	T3914	prep	16,13
R885	T3802	T3798	prep	of,restriction
R886	T3917	T3914	punct	],13
R887	T3918	T3873	punct	.,treated
R888	T3803	T3802	pobj	protein,of
R889	T3920	T3921	mark	While,is
R890	T3921	T3930	advcl	is,known
R891	T3922	T3923	det	the,outcome
R892	T3804	T3803	cc	or,protein
R893	T3923	T3921	nsubj	outcome,is
R894	T3924	T3925	amod	short,term
R895	T3925	T3923	compound	term,outcome
R896	T3805	T3803	conj	BCAA,protein
R897	T3926	T3925	punct	-,term
R898	T3927	T3923	prep	of,outcome
R899	T3928	T3929	compound	liver,transplantation
R900	T3806	T3807	punct	[,8
R901	T3929	T3927	pobj	transplantation,of
R902	T3931	T3921	acomp	encouraging,is
R903	T3807	T3792	parataxis	8,relies
R904	T3932	T3930	punct	", ",known
R905	T3933	T3934	amod	long,term
R906	T3934	T3936	compound	term,effects
R907	T3808	T3807	nummod	1,8
R908	T3935	T3934	punct	-,term
R909	T3936	T3930	nsubjpass	effects,known
R910	T3809	T3807	punct	",",8
R911	T3937	T3936	prep	of,effects
R912	T3938	T3939	det	this,approach
R913	T3939	T3937	pobj	approach,of
R914	T3940	T3930	auxpass	are,known
R915	T3941	T3930	neg	not,known
R916	T3942	T3930	punct	.,known
R917	T3810	T3807	punct	],8
R918	T3944	T3945	advmod	However,required
R919	T3946	T3945	punct	", ",required
R920	T3811	T3792	punct	.,relies
R921	T3947	T3948	det	these,patients
R922	T3948	T3945	nsubjpass	patients,required
R923	T3949	T3945	auxpass	are,required
R924	T3813	T3814	predet	Such,management
R925	T3950	T3945	advmod	now,required
R926	T3814	T3817	nsubj	management,is
R927	T3815	T3814	det	a,management
R928	T3951	T3952	aux	to,take
R929	T3952	T3945	xcomp	take,required
R930	T3953	T3954	compound	immunosuppressant,drugs
R931	T3816	T3814	amod	dietary,management
R932	T3954	T3952	dobj	drugs,take
R933	T3955	T3952	prep	for,take
R934	T3818	T3814	prep	of,management
R935	T3956	T3957	det	the,rest
R936	T3957	T3955	pobj	rest,for
R937	T3958	T3957	prep	of,rest
R938	T3959	T3960	poss	their,lives
R939	T3819	T3820	det	the,disease
R940	T3960	T3958	pobj	lives,of
R941	T3961	T3952	punct	", ",take
R942	T3962	T3963	advmod	often,with
R943	T3820	T3818	pobj	disease,of
R944	T3963	T3952	prep	with,take
R945	T3964	T3965	amod	undesirable,effects
R946	T3965	T3963	pobj	effects,with
R947	T3821	T3817	punct	", ",is
R948	T3966	T3965	compound	side,effects
R949	T3967	T3945	punct	.,required
R950	T3822	T3817	advmod	however,is
R951	T3969	T3970	advmod	Moreover,are
R952	T3823	T3817	punct	", ",is
R953	T3971	T3970	punct	", ",are
R954	T3972	T3973	det	the,cost
R955	T3973	T3970	nsubj	cost,are
R956	T3824	T3817	neg	not,is
R957	T3974	T3973	acl	associated,cost
R958	T3975	T3974	prep	with,associated
R959	T3976	T3977	compound	liver,transplantation
R960	T3825	T3826	advmod	entirely,satisfactory
R961	T3977	T3975	pobj	transplantation,with
R962	T3978	T3973	cc	and,cost
R963	T3979	T3980	det	the,availability
R964	T3980	T3973	conj	availability,cost
R965	T3981	T3980	prep	of,availability
R966	T3826	T3817	acomp	satisfactory,is
R967	T3827	T3828	advmod	especially,in
R968	T3982	T3983	compound	donor,livers
R969	T3828	T3817	prep	in,is
R970	T3983	T3981	pobj	livers,of
R971	T3984	T3985	amod	additional,factors
R972	T3829	T3828	pobj	times,in
R973	T3985	T3970	attr	factors,are
R974	T3986	T3985	amod	limiting,factors
R975	T3987	T3985	prep	for,factors
R976	T3830	T3829	prep	of,times
R977	T3988	T3989	det	the,practicality
R978	T3989	T3987	pobj	practicality,for
R979	T3990	T3989	prep	of,practicality
R980	T3831	T3832	amod	metabolic,decompensation
R981	T3991	T3990	pobj	treatment,of
R982	T3992	T3991	prep	of,treatment
R983	T3832	T3830	pobj	decompensation,of
R984	T3993	T3994	det	this,disease
R985	T3994	T3992	pobj	disease,of
R986	T3833	T3832	prep	due,decompensation
R989	T3834	T3833	pcomp	to,due
R990	T3999	T3997	pcomp	of,Because
R991	T3835	T3833	pobj	infection,due
R992	T4000	T4001	det	the,options
R994	T3836	T3835	punct	", ",infection
R995	T4002	T4001	amod	current,options
R996	T4003	T4001	amod	unsatisfactory,options
R997	T4004	T4001	prep	for,options
R998	T4005	T4006	det	the,treatment
R999	T3837	T3835	conj	injuries,infection
R1000	T4006	T4004	pobj	treatment,for
R1001	T4007	T4006	prep	of,treatment
R1002	T3838	T3837	cc	and,injuries
R1003	T4008	T4007	pobj	MSUD,of
R1004	T4009	T3998	punct	", ",is
R1005	T4010	T3998	expl	there,is
R1006	T3839	T3840	amod	other,stressors
R1007	T4011	T4012	det	a,need
R1008	T4012	T3998	attr	need,is
R1009	T4013	T4012	prep	for,need
R1010	T4014	T4015	amod	improved,therapies
R1011	T3840	T3837	conj	stressors,injuries
R1012	T4015	T4013	pobj	therapies,for
R1013	T4016	T4017	aux	to,combat
R1014	T3841	T3817	punct	.,is
R1015	T4017	T4015	advcl	combat,therapies
R1016	T4018	T4019	det	this,disease
R1017	T4019	T4017	dobj	disease,combat
R1018	T4020	T3998	punct	.,is
R1019	T3843	T3844	prep	In,is
R1020	T4022	T4023	det	An,obstacle
R1021	T4023	T4024	nsubj	obstacle,been
R1022	T4025	T4023	prep	to,obstacle
R1023	T4057	T4058	punct	[,17
R1024	T4026	T4025	pcomp	developing,to
R1025	T4027	T4028	amod	novel,treatments
R1026	T4028	T4026	dobj	treatments,developing
R1027	T4029	T4028	prep	for,treatments
R1028	T4030	T4029	pobj	MSUD,for
R1029	T4031	T4024	aux	has,been
R1030	T4058	T4047	parataxis	17,described
R1031	T4032	T4033	det	the,lack
R1032	T4033	T4024	attr	lack,been
R1033	T4034	T4033	prep	of,lack
R1034	T4059	T4060	punct	-,19
R1035	T4035	T4036	det	a,model
R1036	T4036	T4034	pobj	model,of
R1037	T4037	T4036	amod	suitable,model
R1038	T4038	T4036	compound	animal,model
R1039	T4060	T4058	prep	19,17
R1040	T4039	T4040	aux	to,perform
R1041	T4040	T4036	advcl	perform,model
R1042	T4041	T4042	amod	necessary,studies
R1043	T4061	T4058	punct	],17
R1044	T4042	T4040	dobj	studies,perform
R1045	T4043	T4042	amod	preclinical,studies
R1046	T4044	T4024	punct	.,been
R1047	T4062	T4056	punct	", ",is
R1048	T4046	T4047	mark	Although,described
R1049	T4047	T4056	advcl	described,is
R1050	T4063	T4064	det	this,model
R1051	T4048	T4049	det	a,model
R1052	T4049	T4047	nsubjpass	model,described
R1053	T4050	T4051	compound	Hereford,calf
R1054	T4064	T4056	nsubj	model,is
R1055	T4051	T4049	compound	calf,model
R1056	T4052	T4049	prep	with,model
R1057	T4053	T4052	pobj	MSUD,with
R1058	T4065	T4066	preconj	neither,available
R1059	T4054	T4047	aux	has,described
R1060	T4055	T4047	auxpass	been,described
R1061	T4066	T4056	acomp	available,is
R1062	T4067	T4066	advmod	readily,available
R1063	T4068	T4066	cc	nor,available
R1064	T4069	T4066	conj	practical,available
R1065	T4162	T4156	punct	(,aminotransferase
R1066	T4070	T4071	aux	to,perform
R1067	T4163	T4156	appos	BCAT,aminotransferase
R1068	T4164	T4161	punct	),gene
R1069	T4165	T4154	punct	", ",in
R1070	T4166	T4154	neg	not,in
R1071	T4167	T4154	prep	in,in
R1072	T4071	T4069	xcomp	perform,practical
R1073	T4168	T4167	pobj	BCKDH,in
R1074	T4169	T4168	punct	", ",BCKDH
R1075	T4072	T4073	amod	preclinical,studies
R1076	T4170	T4171	det	the,enzyme
R1077	T4171	T4168	appos	enzyme,BCKDH
R1078	T4172	T4171	amod	deficient,enzyme
R1079	T4173	T4171	prep	in,enzyme
R1080	T4073	T4071	dobj	studies,perform
R1081	T4174	T4173	pobj	MSUD,in
R1082	T4175	T4144	punct	.,disrupts
R1083	T4074	T4056	punct	.,is
R1084	T4177	T4178	mark	Because,is
R1085	T4178	T4181	advcl	is,is
R1086	T4076	T4077	advmod	Furthermore,shown
R1087	T4179	T4180	det	the,mutation
R1088	T4180	T4178	nsubj	mutation,is
R1089	T4182	T4178	neg	not,is
R1090	T4078	T4077	punct	", ",shown
R1091	T4183	T4178	prep	in,is
R1092	T4184	T4183	pobj	BCKDH,in
R1093	T4185	T4181	punct	", ",is
R1094	T4186	T4187	det	the,validity
R1095	T4187	T4181	nsubj	validity,is
R1096	T4079	T4077	nsubj	comparison,shown
R1097	T4188	T4187	prep	of,validity
R1098	T4189	T4190	det	this,line
R1099	T4190	T4188	pobj	line,of
R1100	T4191	T4190	compound	mutant,line
R1101	T4080	T4079	prep	of,comparison
R1102	T4192	T4190	compound	mouse,line
R1103	T4193	T4187	prep	for,validity
R1104	T4194	T4195	amod	modeling,MSUD
R1105	T4081	T4082	det	this,model
R1106	T4195	T4193	pobj	MSUD,for
R1107	T4196	T4195	amod	human,MSUD
R1108	T4197	T4181	acomp	questionable,is
R1109	T4082	T4080	pobj	model,of
R1110	T4198	T4181	punct	.,is
R1111	T4200	T4201	det	The,objective
R1112	T4083	T4082	compound	animal,model
R1113	T4201	T4202	nsubj	objective,was
R1114	T4203	T4201	prep	of,objective
R1115	T4084	T4082	prep	with,model
R1116	T4204	T4205	det	the,study
R1117	T4205	T4203	pobj	study,of
R1118	T4206	T4205	amod	present,study
R1119	T4085	T4086	amod	human,MSUD
R1120	T4207	T4208	aux	to,create
R1121	T4208	T4202	xcomp	create,was
R1122	T4209	T4210	advmod	genetically,engineered
R1123	T4210	T4211	amod	engineered,models
R1124	T4211	T4208	dobj	models,create
R1125	T4212	T4211	amod	murine,models
R1126	T4086	T4084	pobj	MSUD,with
R1127	T4213	T4211	prep	of,models
R1128	T4214	T4213	pobj	MSUD,of
R1129	T4087	T4077	aux	has,shown
R1130	T4215	T4216	dep	that,mimic
R1131	T4216	T4211	relcl	mimic,models
R1132	T4217	T4218	det	the,pathology
R1133	T4088	T4089	det	some,differences
R1134	T4218	T4216	dobj	pathology,mimic
R1135	T4219	T4218	prep	of,pathology
R1136	T4220	T4221	det	the,forms
R1137	T4221	T4219	pobj	forms,of
R1138	T4089	T4077	dobj	differences,shown
R1139	T4222	T4221	amod	classic,forms
R1140	T4223	T4222	cc	and,classic
R1141	T4224	T4222	conj	intermediate,classic
R1142	T4090	T4089	prep	in,differences
R1143	T4225	T4221	amod	variant,forms
R1144	T4226	T4221	prep	of,forms
R1145	T4091	T4092	det	the,pathology
R1146	T4227	T4228	det	the,disease
R1147	T4228	T4226	pobj	disease,of
R1148	T4229	T4228	amod	human,disease
R1149	T4092	T4090	pobj	pathology,in
R1150	T4230	T4202	punct	.,was
R1151	T4232	T4233	det	The,model
R1152	T4093	T4092	prep	of,pathology
R1153	T4233	T4235	nsubjpass	model,created
R1154	T4234	T4233	amod	classic,model
R1155	T4094	T4095	det	the,disease
R1156	T4236	T4235	auxpass	was,created
R1157	T4237	T4235	prep	by,created
R1158	T4095	T4093	pobj	disease,of
R1159	T4238	T4239	amod	targeted,inactivation
R1160	T4239	T4237	pobj	inactivation,by
R1161	T4240	T4239	prep	of,inactivation
R1162	T4096	T4097	punct	[,1
R1163	T4097	T4077	parataxis	1,shown
R1164	T4241	T4242	det	the,subunit
R1165	T4098	T4097	punct	],1
R1166	T4242	T4240	pobj	subunit,of
R1167	T4243	T4242	compound	E2,subunit
R1168	T4099	T4077	punct	", ",shown
R1169	T4244	T4242	prep	of,subunit
R1170	T4245	T4244	pobj	BCKDH,of
R1171	T4246	T4239	prep	by,inactivation
R1172	T4100	T4077	advcl	making,shown
R1173	T4247	T4248	amod	homologous,recombination
R1174	T4248	T4246	pobj	recombination,by
R1175	T4249	T4239	prep	in,inactivation
R1176	T4250	T4251	nmod	embryonic,ES
R1177	T4251	T4254	nmod	ES,cells
R1178	T4101	T4102	det	this,animal
R1179	T4252	T4251	nmod	stem,ES
R1180	T4253	T4251	punct	(,ES
R1181	T4102	T4103	nsubj	animal,model
R1182	T4254	T4249	pobj	cells,in
R1183	T4255	T4254	punct	),cells
R1184	T4256	T4235	punct	.,created
R1185	T4103	T4100	ccomp	model,making
R1186	T4258	T4259	det	The,model
R1187	T4259	T4260	nsubjpass	model,created
R1188	T4104	T4103	det	a,model
R1189	T4261	T4259	prep	of,model
R1190	T4262	T4263	amod	intermediate,MSUD
R1191	T4105	T4106	advmod	less,desirable
R1192	T4263	T4261	pobj	MSUD,of
R1193	T4264	T4260	auxpass	was,created
R1194	T4265	T4260	prep	by,created
R1195	T4106	T4103	amod	desirable,model
R1196	T4266	T4267	amod	partial,rescue
R1197	T4267	T4265	pobj	rescue,by
R1198	T4107	T4103	prep	for,model
R1199	T4108	T4109	det	the,disease
R1200	T4268	T4267	amod	transgenic,rescue
R1201	T4269	T4267	prep	of,rescue
R1202	T4109	T4107	pobj	disease,for
R1203	T4270	T4271	det	the,knockout
R1204	T4271	T4269	pobj	knockout,of
R1205	T4110	T4109	amod	human,disease
R1206	T4272	T4271	compound	E2,knockout
R1207	T4273	T4271	compound	gene,knockout
R1208	T4274	T4260	punct	.,created
R1209	T4111	T4077	punct	.,shown
R1210	T4276	T4277	det	This,report
R1211	T4277	T4278	nsubj	report,describes
R1212	T4113	T4114	advmod	Recently,described
R1213	T4279	T4280	det	the,generation
R1214	T4280	T4278	dobj	generation,describes
R1215	T4281	T4280	cc	and,generation
R1216	T4282	T4280	conj	characterization,generation
R1217	T4115	T4114	punct	", ",described
R1218	T4283	T4280	prep	of,generation
R1219	T4284	T4285	det	these,models
R1220	T4285	T4283	pobj	models,of
R1221	T4286	T4285	amod	murine,models
R1222	T4116	T4117	det	a,nitrosourea
R1223	T4287	T4285	prep	of,models
R1224	T4288	T4287	pobj	MSUD,of
R1225	T4289	T4278	punct	.,describes
R1226	T4291	T4292	det	These,models
R1227	T4117	T4124	npadvmod	nitrosourea,induced
R1228	T4292	T4293	nsubj	models,allow
R1229	T4294	T4293	aux	will,allow
R1230	T4295	T4293	prep	for,allow
R1231	T4118	T4119	compound	N,ethyl
R1232	T4296	T4297	det	the,development
R1233	T4297	T4295	pobj	development,for
R1234	T4298	T4297	prep	of,development
R1235	T4119	T4117	compound	ethyl,nitrosourea
R1236	T4299	T4300	amod	novel,approaches
R1237	T4300	T4298	pobj	approaches,of
R1238	T4301	T4300	compound	treatment,approaches
R1239	T4120	T4119	punct	-,ethyl
R1240	T4302	T4300	punct	", ",approaches
R1241	T4303	T4304	amod	such,as
R1242	T4304	T4300	prep	as,approaches
R1243	T4121	T4117	punct	-,nitrosourea
R1244	T4305	T4306	nmod	gene,therapies
R1245	T4306	T4304	pobj	therapies,as
R1246	T4307	T4305	cc	or,gene
R1247	T4122	T4117	compound	N,nitrosourea
R1248	T4308	T4309	compound	stem,cell
R1249	T4309	T4305	conj	cell,gene
R1250	T4310	T4293	punct	", ",allow
R1251	T4311	T4312	aux	to,cure
R1252	T4123	T4117	punct	-,nitrosourea
R1253	T4312	T4293	advcl	cure,allow
R1254	T4313	T4312	advmod	ultimately,cure
R1255	T4314	T4312	dobj	MSUD,cure
R1256	T4124	T4129	amod	induced,mouse
R1257	T4315	T4293	punct	.,allow
R1258	T4125	T4117	punct	(,nitrosourea
R1259	T4126	T4117	appos	ENU,nitrosourea
R1260	T4127	T4124	punct	),induced
R1261	T4128	T4124	punct	-,induced
R1262	T4129	T4114	nsubjpass	mouse,described
R1263	T4130	T4129	compound	mutant,mouse
R1264	T4131	T4132	dep	that,resembles
R1265	T4132	T4129	relcl	resembles,mouse
R1266	T4133	T4132	advmod	phenotypically,resembles
R1267	T4134	T4135	amod	human,MSUD
R1268	T4135	T4132	dobj	MSUD,resembles
R1269	T4136	T4114	aux	has,described
R1270	T4137	T4114	auxpass	been,described
R1271	T4138	T4139	punct	[,20
R1272	T4139	T4114	parataxis	20,described
R1273	T4140	T4139	punct	],20
R1274	T4141	T4114	punct	.,described
R1275	T4143	T4144	advmod	However,disrupts
R1276	T4145	T4144	punct	", ",disrupts
R1277	T4146	T4147	det	the,mutation
R1278	T4147	T4144	nsubj	mutation,disrupts
R1279	T4148	T4147	prep	in,mutation
R1280	T4149	T4150	det	this,model
R1281	T4150	T4148	pobj	model,in
R1282	T4151	T4152	det	a,site
R1283	T4152	T4144	dobj	site,disrupts
R1284	T4153	T4152	compound	splice,site
R1285	T4154	T4152	prep	in,site
R1286	T4155	T4156	det	the,aminotransferase
R1287	T4156	T4161	nmod	aminotransferase,gene
R1288	T4157	T4156	amod	mitochondrial,aminotransferase
R1289	T4158	T4159	amod	branched,chain
R1290	T4159	T4156	nmod	chain,aminotransferase
R1291	T4160	T4159	punct	-,chain
R1292	T4161	T4154	pobj	gene,in
R643	T3628	T3629	det	The,component
R987	T3995	T3970	punct	.,are
R988	T3997	T3998	prep	Because,is
R993	T4001	T3997	pobj	options,Because

craft-ca-core-ex-dev

Below, discontinuous spans are shown in the chain model. You can change it to the bag model.

Id	Subject	Object	Predicate	Lexical cue
T3189	240-250	CHEBI:25754	denotes	keto acids
T3190	264-290	CHEBI:22918	denotes	branched-chain amino acids
T3191	279-290	CHEBI_SO_EXT:amino_acid	denotes	amino acids
T3192	292-296	CHEBI:22918	denotes	BCAA
T3193	299-306	CHEBI_SO_EXT:leucine	denotes	leucine
T3194	308-318	CHEBI_SO_EXT:isoleucine	denotes	isoleucine
T3195	323-329	CHEBI_SO_EXT:valine	denotes	valine
T3196	391-399	SO_EXT:sequence_alteration_entity_or_process	denotes	mutation
T3197	426-432	CHEBI_GO_SO_EXT:enzyme	denotes	enzyme
T3198	674-678	CHEBI:22918	denotes	BCAA
T3199	682-687	UBERON:0000178	denotes	blood
T3200	698-709	UBERON:0006314	denotes	body fluids
T3201	986-990	CHEBI:22918	denotes	BCAA
T3202	1120-1125	UBERON:0000178	denotes	blood
T3203	1126-1130	CHEBI:22918	denotes	BCAA
T3204	1357-1370	GO:0017086	denotes	BCKDH complex
T3205	1386-1392	CHEBI_GO_SO_EXT:enzyme	denotes	enzyme
T3206	1420-1429	GO_MOP_EXT:catalysis	denotes	catalytic
T3207	1430-1438	CHEBI_PR_EXT:protein	denotes	proteins
T3208	1442-1455	GO_EXT:0016831	denotes	decarboxylase
T3209	1464-1477	CHEBI:50488	denotes	dihydrolipoyl
T3210	1464-1490	GO_PR_EXT:dihydrolipoyllysine_residue_2_methylpropanoyl_transferase	denotes	dihydrolipoyl transacylase
T3211	1492-1494	PR_EXT:000006300	denotes	E2
T3212	1503-1516	CHEBI:50488	denotes	dihydrolipoyl
T3213	1503-1530	GO_PR_EXT:dihydrolipoamide_dehydrogenase	denotes	dihydrolipoyl dehydrogenase
T3214	1532-1534	PR_EXT:000006506	denotes	E3
T3215	1565-1573	GO_EXT:tetrameric_macromolecular_complex	denotes	tetramer
T3216	1590-1591	_FRAGMENT	denotes	α
T3217	1602-1610	PR_EXT:000004686	denotes	subunits
T3218	1600-1610	PR_EXT:000004687	denotes	β subunits
T3219	1627-1629	PR_EXT:000006300	denotes	E2
T3220	1672-1674	PR_EXT:000006506	denotes	E3
T3221	1691-1699	CHEBI:15361	denotes	pyruvate
T3222	1691-1699	_FRAGMENT	denotes	pyruvate
T3223	1720-1733	GO_EXT:0004738	denotes	dehydrogenase
T3224	1720-1743	GO:0045254	denotes	dehydrogenase complexes
T3225	1704-1719	CHEBI_EXT:2_oxoglutarate	denotes	α-ketoglutarate
T3226	1704-1733	GO_EXT:0004591	denotes	α-ketoglutarate dehydrogenase
T3227	1704-1743	GO:0045240	denotes	α-ketoglutarate dehydrogenase complexes
T3228	1756-1763	CHEBI_SO_EXT:glycine	denotes	glycine
T3229	1756-1779	GO:0005960	denotes	glycine cleavage system
T3230	1764-1772	MOP:0000780	denotes	cleavage
T3231	1819-1829	GO:0065007	denotes	regulatory
T3232	1830-1838	CHEBI_PR_EXT:protein	denotes	proteins
T3233	1851-1857	GO_EXT:0016301	denotes	kinase
T3234	1864-1875	GO_EXT:0016791	denotes	phosphatase
T3235	1882-1890	GO:0065007	denotes	regulate
T3236	1912-1918	CHEBI_GO_SO_EXT:enzyme	denotes	enzyme
T3237	1929-1944	GO_PATO_EXT:phosphorylation_process	denotes	phosphorylation
T3238	1964-1981	GO:0016311	denotes	dephosphorylation
T3239	2008-2020	PR_EXT:000004686	denotes	E1 α subunit
T3240	2028-2037	SO_EXT:sequence_alteration_entity_or_process	denotes	Mutations
T3241	2045-2050	SO_EXT:0000704	denotes	genes
T3242	2065-2085	PR_EXT:000006300	denotes	E2 subunits of BCKDH
T3243	2137-2146	SO_EXT:sequence_alteration_entity_or_process	denotes	mutations
T3244	2178-2188	PR_EXT:000006300	denotes	E2 subunit
T3245	2264-2274	GO:0065007	denotes	regulatory
T3246	2275-2281	GO_EXT:0016301	denotes	kinase
T3247	2286-2297	GO_EXT:0016791	denotes	phosphatase
T3248	2390-2397	CHEBI_PR_EXT:protein	denotes	protein
T3249	2401-2405	CHEBI:22918	denotes	BCAA
T3250	2516-2525	GO:0008152	denotes	metabolic
T3251	2780-2785	UBERON:0002107	denotes	liver
T3252	2789-2799	CHEBI_SO_EXT:amino_acid	denotes	amino acid
T3253	2789-2810	GO:0006520	denotes	amino acid metabolism
T3254	2857-2862	NCBITaxon:9606	denotes	human
T3255	2863-2868	UBERON:0002107	denotes	liver
T3256	2928-2933	UBERON:0002107	denotes	liver
T3257	2991-2996	UBERON:0002107	denotes	liver
T3258	3128-3145	CHEBI_EXT:35705	denotes	immunosuppressant
T3259	3146-3151	CHEBI_EXT:23888	denotes	drugs
T3260	3253-3258	UBERON:0002107	denotes	liver
T3261	3305-3311	UBERON:0002107	denotes	livers
T3262	3615-3621	NCBITaxon:33208	denotes	animal
T3263	3690-3694	NCBITaxon_UBERON_EXT:calf	denotes	calf
T3264	3857-3863	NCBITaxon:33208	denotes	animal
T3265	3875-3880	NCBITaxon:9606	denotes	human
T3266	3962-3968	NCBITaxon:33208	denotes	animal
T3267	4000-4005	NCBITaxon:9606	denotes	human
T3268	4027-4048	CHEBI:23995	denotes	N-ethyl-N-nitrosourea
T3269	4050-4053	CHEBI:23995	denotes	ENU
T3270	4063-4069	SO_EXT:sequence_altered_entity_or_alteration_process	denotes	mutant
T3271	4070-4075	NCBITaxon:10088	denotes	mouse
T3272	4106-4111	NCBITaxon:9606	denotes	human
T3273	4155-4163	SO_EXT:sequence_alteration_entity_or_process	denotes	mutation
T3274	4189-4195	GO:0008380	denotes	splice
T3275	4189-4200	SO_EXT:0000162	denotes	splice site
T3276	4208-4221	GO:0005739	denotes	mitochondrial
T3277	4208-4253	PR_EXT:000004683	denotes	mitochondrial branched-chain aminotransferase
T3278	4222-4253	GO_PR_EXT:branched_chain_amino_acid_aminotransferase	denotes	branched-chain aminotransferase
T3279	4255-4259	GO_PR_EXT:branched_chain_amino_acid_aminotransferase	denotes	BCAT
T3280	4261-4265	SO_EXT:0000704	denotes	gene
T3281	4295-4301	CHEBI_GO_SO_EXT:enzyme	denotes	enzyme
T3282	4323-4331	SO_EXT:sequence_alteration_entity_or_process	denotes	mutation
T3283	4370-4376	SO_EXT:sequence_altered_entity_or_alteration_process	denotes	mutant
T3284	4377-4382	NCBITaxon:10088	denotes	mouse
T3285	4401-4406	NCBITaxon:9606	denotes	human
T3286	4478-4489	SO_EXT:0000704	denotes	genetically
T3287	4501-4507	NCBITaxon:39107	denotes	murine
T3288	4601-4606	NCBITaxon:9606	denotes	human
T3289	4678-4697	PR_EXT:000006300	denotes	E2 subunit of BCKDH
T3290	4712-4725	GO_SO_EXT:sequence_rearrangement_process	denotes	recombination
T3181	11-16	NCBITaxon:4022	denotes	Maple
T3182	23-28	UBERON:0001088	denotes	Urine
T3183	49-56	SO_EXT:0000704	denotes	genetic
T3184	107-116	CHEBI:25754	denotes	keto acid
T3185	142-155	GO:0005739	denotes	mitochondrial
T3186	161-167	CHEBI_GO_SO_EXT:enzyme	denotes	enzyme
T3187	168-175	GO:0032991	denotes	complex
T3188	196-205	MOP:0000568	denotes	oxidative
T3291	4729-4738	UBERON:0000922	denotes	embryonic
T3292	4729-4743	_FRAGMENT	denotes	embryonic stem
T3293	4749-4754	CL:0002322	denotes	cells
T3294	4745-4747	_FRAGMENT	denotes	ES
T3295	4749-4754	CL_GO_EXT:cell	denotes	cells
T3296	4810-4820	SO_EXT:transgenic_entity	denotes	transgenic
T3297	4821-4827	SO_EXT:sequence_rescue_process	denotes	rescue
T3298	4835-4837	PR_EXT:000006300	denotes	E2
T3299	4838-4842	SO_EXT:0000704	denotes	gene
T3300	4920-4926	NCBITaxon:39107	denotes	murine
T3301	5026-5030	SO_EXT:0000704	denotes	gene
T3302	5034-5043	CL:0000034	denotes	stem cell
T3303	5039-5043	CL_GO_EXT:cell	denotes	cell
R343	T3217	T3216	_lexicallyChainedTo	subunits,α
R344	T3223	T3222	_lexicallyChainedTo	dehydrogenase,pyruvate
R345	T3224	T3222	_lexicallyChainedTo	dehydrogenase complexes,pyruvate
R346	T3293	T3292	_lexicallyChainedTo	cells,embryonic stem
R347	T3293	T3294	_lexicallyChainedTo	cells,ES

craft-ca-core-dev

Below, discontinuous spans are shown in the chain model. You can change it to the bag model.

Id	Subject	Object	Predicate	Lexical cue
T3120	1532-1534	PR:000006506	denotes	E3
T3123	1600-1610	PR:000004687	denotes	β subunits
T3124	1627-1629	PR:000006300	denotes	E2
T3125	1672-1674	PR:000006506	denotes	E3
T3126	1691-1699	CHEBI:15361	denotes	pyruvate
T3127	1691-1699	_FRAGMENT	denotes	pyruvate
T3128	1720-1743	GO:0045254	denotes	dehydrogenase complexes
T3129	1704-1743	GO:0045240	denotes	α-ketoglutarate dehydrogenase complexes
T3130	1756-1779	GO:0005960	denotes	glycine cleavage system
T3131	1764-1772	MOP:0000780	denotes	cleavage
T3132	1819-1829	GO:0065007	denotes	regulatory
T3133	1882-1890	GO:0065007	denotes	regulate
T3134	1964-1981	GO:0016311	denotes	dephosphorylation
T3135	2008-2020	PR:000004686	denotes	E1 α subunit
T3136	2045-2050	SO:0000704	denotes	genes
T3137	2065-2085	PR:000006300	denotes	E2 subunits of BCKDH
T3138	2178-2188	PR:000006300	denotes	E2 subunit
T3139	2264-2274	GO:0065007	denotes	regulatory
T3140	2401-2405	CHEBI:22918	denotes	BCAA
T3141	2516-2525	GO:0008152	denotes	metabolic
T3142	2780-2785	UBERON:0002107	denotes	liver
T3143	2789-2810	GO:0006520	denotes	amino acid metabolism
T3144	2857-2862	NCBITaxon:9606	denotes	human
T3145	2863-2868	UBERON:0002107	denotes	liver
T3146	2928-2933	UBERON:0002107	denotes	liver
T3147	2991-2996	UBERON:0002107	denotes	liver
T3148	3128-3145	CHEBI:35705	denotes	immunosuppressant
T3149	3146-3151	CHEBI:23888	denotes	drugs
T3150	3253-3258	UBERON:0002107	denotes	liver
T3151	3305-3311	UBERON:0002107	denotes	livers
T3152	3615-3621	NCBITaxon:33208	denotes	animal
T3153	3857-3863	NCBITaxon:33208	denotes	animal
T3154	3875-3880	NCBITaxon:9606	denotes	human
T3155	3962-3968	NCBITaxon:33208	denotes	animal
T3156	4000-4005	NCBITaxon:9606	denotes	human
T3157	4027-4048	CHEBI:23995	denotes	N-ethyl-N-nitrosourea
T3158	4050-4053	CHEBI:23995	denotes	ENU
T3159	4070-4075	NCBITaxon:10088	denotes	mouse
T3160	4106-4111	NCBITaxon:9606	denotes	human
T3161	4189-4195	GO:0008380	denotes	splice
T3162	4189-4200	SO:0000162	denotes	splice site
T3163	4208-4221	GO:0005739	denotes	mitochondrial
T3164	4208-4253	PR:000004683	denotes	mitochondrial branched-chain aminotransferase
T3165	4261-4265	SO:0000704	denotes	gene
T3166	4377-4382	NCBITaxon:10088	denotes	mouse
T3167	4401-4406	NCBITaxon:9606	denotes	human
T3168	4478-4489	SO:0000704	denotes	genetically
T3169	4501-4507	NCBITaxon:39107	denotes	murine
T3170	4601-4606	NCBITaxon:9606	denotes	human
T3171	4678-4697	PR:000006300	denotes	E2 subunit of BCKDH
T3172	4729-4738	UBERON:0000922	denotes	embryonic
T3173	4729-4743	_FRAGMENT	denotes	embryonic stem
T3174	4749-4754	CL:0002322	denotes	cells
T3175	4745-4747	_FRAGMENT	denotes	ES
T3176	4835-4837	PR:000006300	denotes	E2
T3177	4838-4842	SO:0000704	denotes	gene
T3178	4920-4926	NCBITaxon:39107	denotes	murine
T3179	5026-5030	SO:0000704	denotes	gene
T3180	5034-5043	CL:0000034	denotes	stem cell
T3109	292-296	CHEBI:22918	denotes	BCAA
T3110	674-678	CHEBI:22918	denotes	BCAA
T3111	682-687	UBERON:0000178	denotes	blood
T3100	11-16	NCBITaxon:4022	denotes	Maple
T3101	23-28	UBERON:0001088	denotes	Urine
T3102	49-56	SO:0000704	denotes	genetic
T3103	107-116	CHEBI:25754	denotes	keto acid
T3104	142-155	GO:0005739	denotes	mitochondrial
T3105	168-175	GO:0032991	denotes	complex
T3106	196-205	MOP:0000568	denotes	oxidative
T3107	240-250	CHEBI:25754	denotes	keto acids
T3108	264-290	CHEBI:22918	denotes	branched-chain amino acids
T3112	698-709	UBERON:0006314	denotes	body fluids
T3113	986-990	CHEBI:22918	denotes	BCAA
T3114	1120-1125	UBERON:0000178	denotes	blood
T3115	1126-1130	CHEBI:22918	denotes	BCAA
T3116	1357-1370	GO:0017086	denotes	BCKDH complex
T3117	1464-1477	CHEBI:50488	denotes	dihydrolipoyl
T3118	1492-1494	PR:000006300	denotes	E2
T3119	1503-1516	CHEBI:50488	denotes	dihydrolipoyl
T3121	1590-1591	_FRAGMENT	denotes	α
T3122	1602-1610	PR:000004686	denotes	subunits
R339	T3122	T3121	_lexicallyChainedTo	subunits,α
R340	T3128	T3127	_lexicallyChainedTo	dehydrogenase complexes,pyruvate
R341	T3174	T3173	_lexicallyChainedTo	cells,embryonic stem
R342	T3174	T3175	_lexicallyChainedTo	cells,ES

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