Id |
Subject |
Object |
Predicate |
Lexical cue |
T20227 |
1805-1816 |
JJ |
denotes |
biochemical |
T20226 |
1817-1827 |
NNS |
denotes |
phenotypes |
T20225 |
1797-1804 |
JJ |
denotes |
several |
T20224 |
1794-1796 |
IN |
denotes |
to |
T20223 |
1786-1793 |
NN |
denotes |
respect |
T20222 |
1781-1785 |
IN |
denotes |
with |
T20221 |
1767-1772 |
JJ |
denotes |
human |
T20220 |
1773-1780 |
NN |
denotes |
disease |
T20219 |
1763-1766 |
DT |
denotes |
the |
T20218 |
1760-1762 |
IN |
denotes |
of |
T20217 |
1745-1753 |
JJ |
denotes |
faithful |
T20216 |
1754-1759 |
NN |
denotes |
model |
T20215 |
1743-1744 |
DT |
denotes |
a |
T20214 |
1740-1742 |
VB |
denotes |
be |
T20213 |
1737-1739 |
TO |
denotes |
to |
T20212 |
1729-1736 |
VBZ |
denotes |
appears |
T20211 |
1725-1728 |
CC |
denotes |
and |
T20210 |
1720-1724 |
NN |
denotes |
MSUD |
T20209 |
1712-1719 |
JJ |
denotes |
classic |
T20208 |
1709-1711 |
IN |
denotes |
of |
T20207 |
1703-1708 |
NN |
denotes |
model |
T20206 |
1701-1702 |
DT |
denotes |
a |
T20205 |
1679-1689 |
RB |
denotes |
accurately |
T20204 |
1664-1672 |
NN |
denotes |
mutation |
T20203 |
1659-1663 |
JJ |
denotes |
null |
T20202 |
1673-1678 |
NN |
denotes |
mouse |
T20201 |
1655-1658 |
DT |
denotes |
the |
T20200 |
1653-1655 |
, |
denotes |
, |
T20199 |
1650-1653 |
RB |
denotes |
far |
T20198 |
1645-1649 |
RB |
denotes |
thus |
T20197 |
1635-1644 |
VBN |
denotes |
completed |
T20196 |
1610-1626 |
NN |
denotes |
characterization |
T20195 |
1627-1634 |
NNS |
denotes |
studies |
T20194 |
1606-1609 |
DT |
denotes |
the |
T20193 |
1690-1700 |
VBZ |
denotes |
represents |
T20192 |
1601-1605 |
IN |
denotes |
From |
T20191 |
1600-1832 |
sentence |
denotes |
From the characterization studies completed thus far, the null mutation mouse accurately represents a model of classic MSUD and appears to be a faithful model of the human disease with respect to several biochemical phenotypes [1]. |
T20190 |
1599-1600 |
. |
denotes |
. |
T20189 |
1581-1590 |
JJ |
denotes |
enzymatic |
T20188 |
1591-1599 |
NN |
denotes |
activity |
T20187 |
1575-1580 |
NN |
denotes |
BCKDH |
T20186 |
1572-1574 |
IN |
denotes |
of |
T20185 |
1567-1571 |
NN |
denotes |
half |
T20184 |
1553-1566 |
RB |
denotes |
approximately |
T20183 |
1546-1552 |
VBG |
denotes |
having |
T20182 |
1538-1545 |
IN |
denotes |
despite |
T20181 |
1533-1537 |
NN |
denotes |
BCAA |
T20180 |
1530-1532 |
IN |
denotes |
of |
T20179 |
1523-1529 |
NNS |
denotes |
levels |
T20178 |
1516-1522 |
JJ |
denotes |
normal |
T20177 |
1512-1515 |
VBD |
denotes |
had |
T20176 |
1508-1511 |
CC |
denotes |
and |
T20175 |
1501-1507 |
JJ |
denotes |
normal |
T20174 |
1496-1500 |
VBD |
denotes |
were |
T20173 |
1486-1495 |
NNS |
denotes |
knockouts |
T20172 |
1473-1485 |
JJ |
denotes |
Heterozygous |
T20171 |
1472-1600 |
sentence |
denotes |
Heterozygous knockouts were normal and had normal levels of BCAA despite having approximately half of BCKDH enzymatic activity. |
T20170 |
1471-1472 |
. |
denotes |
. |
T20169 |
1470-1471 |
-RRB- |
denotes |
] |
T20168 |
1469-1470 |
CD |
denotes |
1 |
T20167 |
1468-1469 |
-LRB- |
denotes |
[ |
T20166 |
1454-1458 |
NN |
denotes |
MSUD |
T20165 |
1459-1467 |
NNS |
denotes |
patients |
T20164 |
1446-1453 |
JJ |
denotes |
classic |
T20163 |
1443-1445 |
IN |
denotes |
in |
T20162 |
1439-1442 |
CC |
denotes |
and |
T20161 |
1437-1438 |
-RRB- |
denotes |
] |
T20160 |
1435-1437 |
CD |
denotes |
17 |
T20159 |
1434-1435 |
-LRB- |
denotes |
[ |
T20158 |
1424-1428 |
NN |
denotes |
MSUD |
T20157 |
1429-1433 |
NN |
denotes |
calf |
T20156 |
1420-1423 |
DT |
denotes |
the |
T20155 |
1417-1419 |
IN |
denotes |
in |
T20154 |
1408-1416 |
VBN |
denotes |
observed |
T20153 |
1405-1407 |
IN |
denotes |
as |
T20152 |
1392-1404 |
NN |
denotes |
malnutrition |
T20151 |
1388-1391 |
CC |
denotes |
and |
T20150 |
1386-1388 |
, |
denotes |
, |
T20149 |
1375-1386 |
NN |
denotes |
dehydration |
T20148 |
1373-1375 |
, |
denotes |
, |
T20147 |
1368-1373 |
NN |
denotes |
edema |
T20146 |
1362-1367 |
NN |
denotes |
brain |
T20145 |
1360-1362 |
, |
denotes |
, |
T20144 |
1348-1360 |
NN |
denotes |
ketoacidosis |
T20143 |
1346-1348 |
, |
denotes |
, |
T20142 |
1340-1346 |
NNS |
denotes |
levels |
T20141 |
1329-1339 |
JJ |
denotes |
neurotoxic |
T20140 |
1326-1328 |
IN |
denotes |
to |
T20139 |
1321-1325 |
NN |
denotes |
BCAA |
T20138 |
1318-1320 |
IN |
denotes |
of |
T20137 |
1305-1317 |
NN |
denotes |
accumulation |
T20136 |
1301-1304 |
DT |
denotes |
the |
T20135 |
1298-1300 |
IN |
denotes |
to |
T20134 |
1294-1297 |
IN |
denotes |
due |
T20133 |
1287-1293 |
RB |
denotes |
likely |
T20132 |
1284-1286 |
VBZ |
denotes |
is |
T20131 |
1265-1273 |
JJ |
denotes |
neonatal |
T20130 |
1274-1283 |
NN |
denotes |
lethality |
T20129 |
1260-1264 |
DT |
denotes |
This |
T20128 |
1259-1472 |
sentence |
denotes |
This neonatal lethality is likely due to the accumulation of BCAA to neurotoxic levels, ketoacidosis, brain edema, dehydration, and malnutrition as observed in the MSUD calf [17] and in classic MSUD patients [1]. |
T20127 |
1258-1259 |
. |
denotes |
. |
T20126 |
1253-1258 |
NN |
denotes |
birth |
T20125 |
1250-1252 |
IN |
denotes |
of |
T20124 |
1244-1249 |
NNS |
denotes |
hours |
T20123 |
1241-1243 |
CD |
denotes |
72 |
T20122 |
1234-1240 |
IN |
denotes |
within |
T20121 |
1213-1223 |
JJ |
denotes |
homozygous |
T20120 |
1224-1228 |
NNS |
denotes |
pups |
T20119 |
1209-1212 |
DT |
denotes |
the |
T20118 |
1206-1208 |
IN |
denotes |
of |
T20117 |
1202-1205 |
DT |
denotes |
all |
T20116 |
1195-1201 |
RB |
denotes |
nearly |
T20115 |
1193-1195 |
, |
denotes |
, |
T20114 |
1229-1233 |
VBD |
denotes |
died |
T20113 |
1186-1193 |
RB |
denotes |
Finally |
T20112 |
1185-1259 |
sentence |
denotes |
Finally, nearly all of the homozygous pups died within 72 hours of birth. |
T20111 |
1184-1185 |
. |
denotes |
. |
T20110 |
1183-1184 |
-RRB- |
denotes |
] |
T20109 |
1180-1181 |
, |
denotes |
, |
T20108 |
1179-1180 |
CD |
denotes |
1 |
T20107 |
1181-1183 |
CD |
denotes |
37 |
T20106 |
1178-1179 |
-LRB- |
denotes |
[ |
T20105 |
1169-1177 |
NNS |
denotes |
patients |
T20104 |
1166-1168 |
IN |
denotes |
in |
T20103 |
1149-1165 |
NNS |
denotes |
encephalopathies |
T20102 |
1144-1148 |
NN |
denotes |
MSUD |
T20101 |
1140-1143 |
IN |
denotes |
for |
T20100 |
1132-1139 |
NN |
denotes |
culprit |
T20099 |
1128-1131 |
DT |
denotes |
the |
T20098 |
1113-1116 |
VBP |
denotes |
are |
T20097 |
1117-1127 |
VBN |
denotes |
considered |
T20096 |
1107-1112 |
WDT |
denotes |
which |
T20095 |
1105-1107 |
, |
denotes |
, |
T20094 |
1094-1099 |
NN |
denotes |
amino |
T20093 |
1082-1093 |
JJ |
denotes |
neuroactive |
T20092 |
1074-1081 |
JJ |
denotes |
similar |
T20091 |
1100-1105 |
NNS |
denotes |
acids |
T20090 |
1068-1073 |
JJ |
denotes |
other |
T20089 |
1064-1067 |
CC |
denotes |
and |
T20088 |
1062-1064 |
, |
denotes |
, |
T20087 |
1055-1062 |
NN |
denotes |
alanine |
T20086 |
1053-1055 |
, |
denotes |
, |
T20085 |
1044-1053 |
NN |
denotes |
glutamine |
T20084 |
1042-1044 |
, |
denotes |
, |
T20083 |
1033-1042 |
NN |
denotes |
glutamate |
T20082 |
1030-1032 |
IN |
denotes |
of |
T20081 |
1023-1029 |
NNS |
denotes |
levels |
T20080 |
1015-1022 |
VBN |
denotes |
reduced |
T20079 |
1010-1014 |
IN |
denotes |
from |
T20078 |
999-1002 |
MD |
denotes |
may |
T20077 |
1003-1009 |
VB |
denotes |
result |
T20076 |
979-989 |
JJ |
denotes |
neurologic |
T20075 |
990-998 |
NNS |
denotes |
symptoms |
T20074 |
973-978 |
DT |
denotes |
These |
T20073 |
972-1185 |
sentence |
denotes |
These neurologic symptoms may result from reduced levels of glutamate, glutamine, alanine, and other similar neuroactive amino acids, which are considered the culprit for MSUD encephalopathies in patients [1,37]. |
T20072 |
971-972 |
. |
denotes |
. |
T20071 |
970-971 |
-RRB- |
denotes |
] |
T20070 |
969-970 |
CD |
denotes |
1 |
T20069 |
968-969 |
-LRB- |
denotes |
[ |
T20068 |
955-967 |
NNS |
denotes |
difficulties |
T20067 |
943-954 |
JJ |
denotes |
respiratory |
T20066 |
939-942 |
CC |
denotes |
and |
T20065 |
937-939 |
, |
denotes |
, |
T20064 |
929-937 |
NN |
denotes |
activity |
T20063 |
923-928 |
NN |
denotes |
motor |
T20062 |
920-922 |
IN |
denotes |
of |
T20061 |
915-919 |
NN |
denotes |
loss |
T20060 |
913-915 |
, |
denotes |
, |
T20059 |
905-913 |
NN |
denotes |
lethargy |
T20058 |
903-905 |
, |
denotes |
, |
T20057 |
897-903 |
NN |
denotes |
stupor |
T20056 |
895-897 |
, |
denotes |
, |
T20055 |
887-895 |
NNS |
denotes |
seizures |
T20054 |
884-886 |
IN |
denotes |
as |
T20053 |
879-883 |
JJ |
denotes |
such |
T20052 |
867-878 |
NN |
denotes |
dysfunction |
T20051 |
856-866 |
JJ |
denotes |
neurologic |
T20050 |
853-855 |
IN |
denotes |
of |
T20049 |
847-852 |
NNS |
denotes |
signs |
T20048 |
839-846 |
VBP |
denotes |
include |
T20047 |
833-838 |
DT |
denotes |
These |
T20046 |
832-972 |
sentence |
denotes |
These include signs of neurologic dysfunction such as seizures, stupor, lethargy, loss of motor activity, and respiratory difficulties [1]. |
T20045 |
831-832 |
. |
denotes |
. |
T20044 |
818-822 |
NN |
denotes |
MSUD |
T20043 |
810-817 |
JJ |
denotes |
classic |
T20042 |
823-831 |
NNS |
denotes |
patients |
T20041 |
802-809 |
JJ |
denotes |
newborn |
T20040 |
799-801 |
IN |
denotes |
in |
T20039 |
794-798 |
VBN |
denotes |
seen |
T20038 |
785-793 |
NNS |
denotes |
symptoms |
T20037 |
764-769 |
NN |
denotes |
mouse |
T20036 |
770-774 |
NNS |
denotes |
pups |
T20035 |
753-763 |
JJ |
denotes |
homozygous |
T20034 |
750-752 |
IN |
denotes |
of |
T20033 |
775-784 |
VBZ |
denotes |
resembles |
T20032 |
730-740 |
JJ |
denotes |
phenotypic |
T20031 |
741-749 |
NN |
denotes |
behavior |
T20030 |
726-729 |
DT |
denotes |
The |
T20029 |
725-832 |
sentence |
denotes |
The phenotypic behavior of homozygous mouse pups resembles symptoms seen in newborn classic MSUD patients. |
T20028 |
724-725 |
. |
denotes |
. |
T20027 |
720-724 |
NNS |
denotes |
pups |
T20026 |
709-719 |
JJ |
denotes |
homozygous |
T20025 |
706-708 |
IN |
denotes |
of |
T20024 |
694-705 |
NNS |
denotes |
fibroblasts |
T20023 |
690-693 |
CC |
denotes |
and |
T20022 |
684-689 |
NN |
denotes |
liver |
T20021 |
681-683 |
IN |
denotes |
in |
T20020 |
674-680 |
JJ |
denotes |
absent |
T20019 |
670-673 |
VBD |
denotes |
was |
T20018 |
659-661 |
NN |
denotes |
E2 |
T20017 |
662-669 |
NN |
denotes |
protein |
T20016 |
644-658 |
JJ |
denotes |
Immunoreactive |
T20015 |
643-725 |
sentence |
denotes |
Immunoreactive E2 protein was absent in liver and fibroblasts of homozygous pups. |
T20014 |
642-643 |
. |
denotes |
. |
T20013 |
638-642 |
NN |
denotes |
BCAA |
T20012 |
624-637 |
JJ |
denotes |
unmetabolized |
T19940 |
247-252 |
NN |
denotes |
birth |
T19939 |
244-246 |
IN |
denotes |
at |
T19938 |
237-243 |
JJ |
denotes |
normal |
T19937 |
228-236 |
VBD |
denotes |
appeared |
T19936 |
224-227 |
CC |
denotes |
and |
T19935 |
205-213 |
VBN |
denotes |
expected |
T19934 |
214-223 |
NN |
denotes |
frequency |
T19933 |
201-204 |
DT |
denotes |
the |
T19932 |
198-200 |
IN |
denotes |
at |
T19931 |
188-192 |
VBD |
denotes |
were |
T19930 |
193-197 |
VBN |
denotes |
born |
T19929 |
183-187 |
NNS |
denotes |
mice |
T19928 |
174-182 |
NN |
denotes |
Knockout |
T19927 |
173-253 |
sentence |
denotes |
Knockout mice were born at the expected frequency and appeared normal at birth. |
T19926 |
172-173 |
. |
denotes |
. |
T19925 |
168-172 |
NN |
denotes |
MSUD |
T19924 |
160-167 |
JJ |
denotes |
classic |
T19923 |
155-159 |
IN |
denotes |
with |
T19922 |
148-154 |
NNS |
denotes |
humans |
T19921 |
145-147 |
IN |
denotes |
to |
T19920 |
137-144 |
JJ |
denotes |
similar |
T19919 |
126-136 |
RB |
denotes |
strikingly |
T19918 |
106-114 |
NN |
denotes |
knockout |
T19917 |
115-122 |
NNS |
denotes |
animals |
T19916 |
100-105 |
DT |
denotes |
these |
T19915 |
97-99 |
IN |
denotes |
of |
T19914 |
123-125 |
VBZ |
denotes |
is |
T19913 |
87-96 |
NN |
denotes |
phenotype |
T19912 |
83-86 |
DT |
denotes |
The |
T19911 |
82-173 |
sentence |
denotes |
The phenotype of these knockout animals is strikingly similar to humans with classic MSUD. |
T19910 |
81-82 |
. |
denotes |
. |
T19909 |
76-81 |
NN |
denotes |
BCKDH |
T19908 |
73-75 |
IN |
denotes |
of |
T19907 |
62-64 |
NN |
denotes |
E2 |
T19906 |
65-72 |
NN |
denotes |
subunit |
T19905 |
58-61 |
DT |
denotes |
the |
T19904 |
55-57 |
IN |
denotes |
of |
T19903 |
46-54 |
NN |
denotes |
knockout |
T19902 |
41-45 |
NN |
denotes |
gene |
T19901 |
38-40 |
IN |
denotes |
by |
T19900 |
26-29 |
VBD |
denotes |
was |
T19899 |
21-25 |
NN |
denotes |
MSUD |
T19898 |
13-20 |
JJ |
denotes |
classic |
T19897 |
10-12 |
IN |
denotes |
of |
T19896 |
30-37 |
VBN |
denotes |
created |
T19895 |
4-9 |
NN |
denotes |
model |
T19894 |
0-3 |
PRP$ |
denotes |
Our |
T20231 |
1831-1832 |
. |
denotes |
. |
T20230 |
1830-1831 |
-RRB- |
denotes |
] |
T20229 |
1829-1830 |
CD |
denotes |
1 |
T20228 |
1828-1829 |
-LRB- |
denotes |
[ |
T20011 |
621-623 |
IN |
denotes |
of |
T20010 |
608-620 |
NN |
denotes |
accumulation |
T20009 |
604-607 |
DT |
denotes |
the |
T20008 |
600-603 |
IN |
denotes |
for |
T20007 |
589-599 |
VBG |
denotes |
accounting |
T20006 |
587-589 |
, |
denotes |
, |
T20005 |
575-587 |
JJ |
denotes |
undetectable |
T20004 |
560-565 |
NN |
denotes |
mouse |
T20003 |
566-570 |
NNS |
denotes |
pups |
T20002 |
551-559 |
NN |
denotes |
knockout |
T20001 |
540-550 |
JJ |
denotes |
homozygous |
T20000 |
537-539 |
IN |
denotes |
of |
T19999 |
530-536 |
NNS |
denotes |
livers |
T19998 |
527-529 |
IN |
denotes |
in |
T19997 |
521-526 |
NN |
denotes |
BCKDH |
T19996 |
518-520 |
IN |
denotes |
of |
T19995 |
571-574 |
VBD |
denotes |
was |
T19994 |
509-517 |
NN |
denotes |
activity |
T19993 |
505-508 |
DT |
denotes |
The |
T19992 |
504-643 |
sentence |
denotes |
The activity of BCKDH in livers of homozygous knockout mouse pups was undetectable, accounting for the accumulation of unmetabolized BCAA. |
T19991 |
503-504 |
. |
denotes |
. |
T19990 |
502-503 |
-RRB- |
denotes |
) |
T19989 |
501-502 |
CD |
denotes |
1 |
T19988 |
495-500 |
NN |
denotes |
Table |
T19987 |
494-495 |
-LRB- |
denotes |
( |
T19986 |
479-483 |
VBD |
denotes |
were |
T19985 |
477-479 |
, |
denotes |
, |
T19984 |
473-477 |
NN |
denotes |
BCAA |
T19983 |
470-472 |
IN |
denotes |
of |
T19982 |
459-469 |
NN |
denotes |
metabolism |
T19981 |
452-458 |
JJ |
denotes |
normal |
T19980 |
447-451 |
IN |
denotes |
with |
T19979 |
437-439 |
VBZ |
denotes |
is |
T19978 |
440-446 |
VBN |
denotes |
linked |
T19977 |
427-436 |
NN |
denotes |
synthesis |
T19976 |
421-426 |
WP$ |
denotes |
whose |
T19975 |
419-421 |
, |
denotes |
, |
T19974 |
410-419 |
NN |
denotes |
glutamine |
T19973 |
406-409 |
CC |
denotes |
and |
T19972 |
404-406 |
, |
denotes |
, |
T19971 |
395-404 |
NN |
denotes |
glutamate |
T19970 |
393-395 |
, |
denotes |
, |
T19969 |
386-393 |
NN |
denotes |
alanine |
T19968 |
374-379 |
NN |
denotes |
amino |
T19967 |
380-385 |
NNS |
denotes |
acids |
T19966 |
370-373 |
DT |
denotes |
the |
T19965 |
367-369 |
IN |
denotes |
of |
T19964 |
360-366 |
NNS |
denotes |
levels |
T19963 |
358-360 |
, |
denotes |
, |
T19962 |
484-493 |
VBN |
denotes |
decreased |
T19961 |
345-358 |
RB |
denotes |
Concomitantly |
T19960 |
344-504 |
sentence |
denotes |
Concomitantly, levels of the amino acids alanine, glutamate, and glutamine, whose synthesis is linked with normal metabolism of BCAA, were decreased (Table 1). |
T19959 |
343-344 |
. |
denotes |
. |
T19958 |
335-343 |
JJ |
denotes |
elevated |
T19957 |
326-334 |
RB |
denotes |
markedly |
T19956 |
316-320 |
NN |
denotes |
BCAA |
T19955 |
313-315 |
IN |
denotes |
of |
T19954 |
306-312 |
NNS |
denotes |
levels |
T19953 |
300-305 |
NN |
denotes |
blood |
T19952 |
298-300 |
, |
denotes |
, |
T19951 |
290-298 |
NN |
denotes |
suckling |
T19950 |
280-289 |
VBG |
denotes |
following |
T19949 |
276-279 |
CC |
denotes |
and |
T19948 |
270-275 |
NN |
denotes |
birth |
T19947 |
267-269 |
IN |
denotes |
of |
T19946 |
263-266 |
NN |
denotes |
day |
T19945 |
261-262 |
DT |
denotes |
a |
T19944 |
321-325 |
VBD |
denotes |
were |
T19943 |
254-260 |
IN |
denotes |
Within |
T19942 |
253-344 |
sentence |
denotes |
Within a day of birth and following suckling, blood levels of BCAA were markedly elevated. |
T19941 |
252-253 |
. |
denotes |
. |
R5030 |
T19894 |
T19895 |
poss |
Our,model |
R5031 |
T19895 |
T19896 |
nsubjpass |
model,created |
R5032 |
T19897 |
T19895 |
prep |
of,model |
R5033 |
T19898 |
T19899 |
amod |
classic,MSUD |
R5034 |
T19899 |
T19897 |
pobj |
MSUD,of |
R5035 |
T19900 |
T19896 |
auxpass |
was,created |
R5036 |
T19901 |
T19896 |
prep |
by,created |
R5037 |
T19902 |
T19903 |
compound |
gene,knockout |
R5038 |
T19903 |
T19901 |
pobj |
knockout,by |
R5039 |
T19904 |
T19903 |
prep |
of,knockout |
R5040 |
T19905 |
T19906 |
det |
the,subunit |
R5041 |
T19906 |
T19904 |
pobj |
subunit,of |
R5042 |
T19907 |
T19906 |
compound |
E2,subunit |
R5043 |
T19908 |
T19906 |
prep |
of,subunit |
R5044 |
T19909 |
T19908 |
pobj |
BCKDH,of |
R5045 |
T19910 |
T19896 |
punct |
.,created |
R5046 |
T19912 |
T19913 |
det |
The,phenotype |
R5047 |
T19913 |
T19914 |
nsubj |
phenotype,is |
R5048 |
T19915 |
T19913 |
prep |
of,phenotype |
R5049 |
T19916 |
T19917 |
det |
these,animals |
R5050 |
T19917 |
T19915 |
pobj |
animals,of |
R5051 |
T19918 |
T19917 |
compound |
knockout,animals |
R5052 |
T19919 |
T19920 |
advmod |
strikingly,similar |
R5053 |
T19920 |
T19914 |
acomp |
similar,is |
R5054 |
T19921 |
T19920 |
prep |
to,similar |
R5055 |
T19922 |
T19921 |
pobj |
humans,to |
R5056 |
T19923 |
T19922 |
prep |
with,humans |
R5057 |
T19924 |
T19925 |
amod |
classic,MSUD |
R5058 |
T19925 |
T19923 |
pobj |
MSUD,with |
R5059 |
T19926 |
T19914 |
punct |
.,is |
R5060 |
T19928 |
T19929 |
compound |
Knockout,mice |
R5061 |
T19929 |
T19930 |
nsubjpass |
mice,born |
R5062 |
T19931 |
T19930 |
auxpass |
were,born |
R5063 |
T19932 |
T19930 |
prep |
at,born |
R5064 |
T19933 |
T19934 |
det |
the,frequency |
R5065 |
T19934 |
T19932 |
pobj |
frequency,at |
R5066 |
T19935 |
T19934 |
amod |
expected,frequency |
R5067 |
T19936 |
T19930 |
cc |
and,born |
R5068 |
T19937 |
T19930 |
conj |
appeared,born |
R5069 |
T19938 |
T19937 |
oprd |
normal,appeared |
R5070 |
T19939 |
T19937 |
prep |
at,appeared |
R5071 |
T19940 |
T19939 |
pobj |
birth,at |
R5072 |
T19941 |
T19930 |
punct |
.,born |
R5073 |
T19943 |
T19944 |
prep |
Within,were |
R5074 |
T19945 |
T19946 |
det |
a,day |
R5075 |
T19946 |
T19943 |
pobj |
day,Within |
R5076 |
T19947 |
T19946 |
prep |
of,day |
R5077 |
T19948 |
T19947 |
pobj |
birth,of |
R5078 |
T19949 |
T19943 |
cc |
and,Within |
R5079 |
T19950 |
T19943 |
conj |
following,Within |
R5080 |
T19951 |
T19950 |
pobj |
suckling,following |
R5081 |
T19952 |
T19944 |
punct |
", ",were |
R5082 |
T19953 |
T19954 |
compound |
blood,levels |
R5083 |
T19954 |
T19944 |
nsubj |
levels,were |
R5084 |
T19955 |
T19954 |
prep |
of,levels |
R5085 |
T19956 |
T19955 |
pobj |
BCAA,of |
R5086 |
T19957 |
T19958 |
advmod |
markedly,elevated |
R5087 |
T19958 |
T19944 |
acomp |
elevated,were |
R5088 |
T19959 |
T19944 |
punct |
.,were |
R5089 |
T19961 |
T19962 |
advmod |
Concomitantly,decreased |
R5090 |
T19963 |
T19962 |
punct |
", ",decreased |
R5091 |
T19964 |
T19962 |
nsubjpass |
levels,decreased |
R5092 |
T19965 |
T19964 |
prep |
of,levels |
R5093 |
T19966 |
T19967 |
det |
the,acids |
R5094 |
T19967 |
T19965 |
pobj |
acids,of |
R5095 |
T19968 |
T19967 |
compound |
amino,acids |
R5096 |
T19969 |
T19967 |
appos |
alanine,acids |
R5097 |
T19970 |
T19969 |
punct |
", ",alanine |
R5098 |
T19971 |
T19969 |
conj |
glutamate,alanine |
R5099 |
T19972 |
T19971 |
punct |
", ",glutamate |
R5100 |
T19973 |
T19971 |
cc |
and,glutamate |
R5101 |
T19974 |
T19971 |
conj |
glutamine,glutamate |
R5102 |
T19975 |
T19967 |
punct |
", ",acids |
R5103 |
T19976 |
T19977 |
poss |
whose,synthesis |
R5104 |
T19977 |
T19978 |
dep |
synthesis,linked |
R5105 |
T19978 |
T19967 |
relcl |
linked,acids |
R5106 |
T19979 |
T19978 |
auxpass |
is,linked |
R5107 |
T19980 |
T19978 |
prep |
with,linked |
R5108 |
T19981 |
T19982 |
amod |
normal,metabolism |
R5109 |
T19982 |
T19980 |
pobj |
metabolism,with |
R5110 |
T19983 |
T19982 |
prep |
of,metabolism |
R5111 |
T19984 |
T19983 |
pobj |
BCAA,of |
R5112 |
T19985 |
T19962 |
punct |
", ",decreased |
R5113 |
T19986 |
T19962 |
auxpass |
were,decreased |
R5114 |
T19987 |
T19988 |
punct |
(,Table |
R5115 |
T19988 |
T19962 |
parataxis |
Table,decreased |
R5116 |
T19989 |
T19988 |
nummod |
1,Table |
R5117 |
T19990 |
T19988 |
punct |
),Table |
R5118 |
T19991 |
T19962 |
punct |
.,decreased |
R5119 |
T19993 |
T19994 |
det |
The,activity |
R5120 |
T19994 |
T19995 |
nsubj |
activity,was |
R5121 |
T19996 |
T19994 |
prep |
of,activity |
R5122 |
T19997 |
T19996 |
pobj |
BCKDH,of |
R5123 |
T19998 |
T19994 |
prep |
in,activity |
R5124 |
T19999 |
T19998 |
pobj |
livers,in |
R5125 |
T20000 |
T19999 |
prep |
of,livers |
R5126 |
T20001 |
T20002 |
amod |
homozygous,knockout |
R5127 |
T20002 |
T20003 |
compound |
knockout,pups |
R5128 |
T20003 |
T20000 |
pobj |
pups,of |
R5129 |
T20004 |
T20003 |
compound |
mouse,pups |
R5130 |
T20005 |
T19995 |
acomp |
undetectable,was |
R5131 |
T20006 |
T19995 |
punct |
", ",was |
R5132 |
T20007 |
T19995 |
advcl |
accounting,was |
R5133 |
T20008 |
T20007 |
prep |
for,accounting |
R5134 |
T20009 |
T20010 |
det |
the,accumulation |
R5135 |
T20010 |
T20008 |
pobj |
accumulation,for |
R5136 |
T20011 |
T20010 |
prep |
of,accumulation |
R5137 |
T20012 |
T20013 |
amod |
unmetabolized,BCAA |
R5138 |
T20013 |
T20011 |
pobj |
BCAA,of |
R5139 |
T20014 |
T19995 |
punct |
.,was |
R5140 |
T20016 |
T20017 |
amod |
Immunoreactive,protein |
R5141 |
T20017 |
T20019 |
nsubj |
protein,was |
R5142 |
T20018 |
T20017 |
compound |
E2,protein |
R5143 |
T20020 |
T20019 |
acomp |
absent,was |
R5144 |
T20021 |
T20019 |
prep |
in,was |
R5145 |
T20022 |
T20021 |
pobj |
liver,in |
R5146 |
T20023 |
T20022 |
cc |
and,liver |
R5147 |
T20024 |
T20022 |
conj |
fibroblasts,liver |
R5148 |
T20025 |
T20022 |
prep |
of,liver |
R5149 |
T20026 |
T20027 |
amod |
homozygous,pups |
R5150 |
T20027 |
T20025 |
pobj |
pups,of |
R5151 |
T20028 |
T20019 |
punct |
.,was |
R5152 |
T20030 |
T20031 |
det |
The,behavior |
R5153 |
T20031 |
T20033 |
nsubj |
behavior,resembles |
R5154 |
T20032 |
T20031 |
amod |
phenotypic,behavior |
R5155 |
T20034 |
T20031 |
prep |
of,behavior |
R5156 |
T20035 |
T20036 |
amod |
homozygous,pups |
R5157 |
T20036 |
T20034 |
pobj |
pups,of |
R5158 |
T20037 |
T20036 |
compound |
mouse,pups |
R5159 |
T20038 |
T20033 |
dobj |
symptoms,resembles |
R5160 |
T20039 |
T20038 |
acl |
seen,symptoms |
R5161 |
T20040 |
T20039 |
prep |
in,seen |
R5162 |
T20041 |
T20042 |
amod |
newborn,patients |
R5163 |
T20042 |
T20040 |
pobj |
patients,in |
R5164 |
T20043 |
T20042 |
amod |
classic,patients |
R5165 |
T20044 |
T20042 |
compound |
MSUD,patients |
R5166 |
T20045 |
T20033 |
punct |
.,resembles |
R5167 |
T20047 |
T20048 |
nsubj |
These,include |
R5168 |
T20049 |
T20048 |
dobj |
signs,include |
R5169 |
T20050 |
T20049 |
prep |
of,signs |
R5170 |
T20051 |
T20052 |
amod |
neurologic,dysfunction |
R5171 |
T20052 |
T20050 |
pobj |
dysfunction,of |
R5172 |
T20053 |
T20054 |
amod |
such,as |
R5173 |
T20054 |
T20049 |
prep |
as,signs |
R5174 |
T20055 |
T20054 |
pobj |
seizures,as |
R5175 |
T20056 |
T20055 |
punct |
", ",seizures |
R5176 |
T20057 |
T20055 |
conj |
stupor,seizures |
R5177 |
T20058 |
T20057 |
punct |
", ",stupor |
R5178 |
T20059 |
T20057 |
conj |
lethargy,stupor |
R5179 |
T20060 |
T20059 |
punct |
", ",lethargy |
R5180 |
T20061 |
T20059 |
conj |
loss,lethargy |
R5181 |
T20062 |
T20061 |
prep |
of,loss |
R5182 |
T20063 |
T20064 |
compound |
motor,activity |
R5183 |
T20064 |
T20062 |
pobj |
activity,of |
R5184 |
T20065 |
T20061 |
punct |
", ",loss |
R5185 |
T20066 |
T20061 |
cc |
and,loss |
R5186 |
T20067 |
T20068 |
amod |
respiratory,difficulties |
R5187 |
T20068 |
T20061 |
conj |
difficulties,loss |
R5188 |
T20069 |
T20070 |
punct |
[,1 |
R5189 |
T20070 |
T20048 |
parataxis |
1,include |
R5190 |
T20071 |
T20070 |
punct |
],1 |
R5191 |
T20072 |
T20048 |
punct |
.,include |
R5192 |
T20074 |
T20075 |
det |
These,symptoms |
R5193 |
T20075 |
T20077 |
nsubj |
symptoms,result |
R5194 |
T20076 |
T20075 |
amod |
neurologic,symptoms |
R5195 |
T20078 |
T20077 |
aux |
may,result |
R5196 |
T20079 |
T20077 |
prep |
from,result |
R5197 |
T20080 |
T20081 |
amod |
reduced,levels |
R5198 |
T20081 |
T20079 |
pobj |
levels,from |
R5199 |
T20082 |
T20081 |
prep |
of,levels |
R5200 |
T20083 |
T20082 |
pobj |
glutamate,of |
R5201 |
T20084 |
T20083 |
punct |
", ",glutamate |
R5202 |
T20085 |
T20083 |
conj |
glutamine,glutamate |
R5203 |
T20086 |
T20085 |
punct |
", ",glutamine |
R5204 |
T20087 |
T20085 |
conj |
alanine,glutamine |
R5205 |
T20088 |
T20087 |
punct |
", ",alanine |
R5206 |
T20089 |
T20087 |
cc |
and,alanine |
R5207 |
T20090 |
T20091 |
amod |
other,acids |
R5208 |
T20091 |
T20087 |
conj |
acids,alanine |
R5209 |
T20092 |
T20091 |
amod |
similar,acids |
R5210 |
T20093 |
T20091 |
amod |
neuroactive,acids |
R5211 |
T20094 |
T20091 |
compound |
amino,acids |
R5212 |
T20095 |
T20083 |
punct |
", ",glutamate |
R5213 |
T20096 |
T20097 |
dep |
which,considered |
R5214 |
T20097 |
T20083 |
relcl |
considered,glutamate |
R5215 |
T20098 |
T20097 |
auxpass |
are,considered |
R5216 |
T20099 |
T20100 |
det |
the,culprit |
R5217 |
T20100 |
T20097 |
oprd |
culprit,considered |
R5218 |
T20101 |
T20100 |
prep |
for,culprit |
R5219 |
T20102 |
T20103 |
compound |
MSUD,encephalopathies |
R5220 |
T20103 |
T20101 |
pobj |
encephalopathies,for |
R5221 |
T20104 |
T20100 |
prep |
in,culprit |
R5222 |
T20105 |
T20104 |
pobj |
patients,in |
R5223 |
T20106 |
T20107 |
punct |
[,37 |
R5224 |
T20107 |
T20077 |
parataxis |
37,result |
R5225 |
T20108 |
T20107 |
nummod |
1,37 |
R5226 |
T20109 |
T20107 |
punct |
",",37 |
R5227 |
T20110 |
T20107 |
punct |
],37 |
R5228 |
T20111 |
T20077 |
punct |
.,result |
R5229 |
T20113 |
T20114 |
advmod |
Finally,died |
R5230 |
T20115 |
T20114 |
punct |
", ",died |
R5231 |
T20116 |
T20117 |
advmod |
nearly,all |
R5232 |
T20117 |
T20114 |
nsubj |
all,died |
R5233 |
T20118 |
T20117 |
prep |
of,all |
R5234 |
T20119 |
T20120 |
det |
the,pups |
R5235 |
T20120 |
T20118 |
pobj |
pups,of |
R5236 |
T20121 |
T20120 |
amod |
homozygous,pups |
R5237 |
T20122 |
T20114 |
prep |
within,died |
R5238 |
T20123 |
T20124 |
nummod |
72,hours |
R5239 |
T20124 |
T20122 |
pobj |
hours,within |
R5240 |
T20125 |
T20124 |
prep |
of,hours |
R5241 |
T20126 |
T20125 |
pobj |
birth,of |
R5242 |
T20127 |
T20114 |
punct |
.,died |
R5243 |
T20129 |
T20130 |
det |
This,lethality |
R5244 |
T20130 |
T20132 |
nsubj |
lethality,is |
R5245 |
T20131 |
T20130 |
amod |
neonatal,lethality |
R5246 |
T20133 |
T20134 |
advmod |
likely,due |
R5247 |
T20134 |
T20132 |
prep |
due,is |
R5248 |
T20135 |
T20134 |
pcomp |
to,due |
R5249 |
T20136 |
T20137 |
det |
the,accumulation |
R5250 |
T20137 |
T20134 |
pobj |
accumulation,due |
R5251 |
T20138 |
T20137 |
prep |
of,accumulation |
R5252 |
T20139 |
T20138 |
pobj |
BCAA,of |
R5253 |
T20140 |
T20137 |
prep |
to,accumulation |
R5254 |
T20141 |
T20142 |
amod |
neurotoxic,levels |
R5255 |
T20142 |
T20140 |
pobj |
levels,to |
R5256 |
T20143 |
T20142 |
punct |
", ",levels |
R5257 |
T20144 |
T20142 |
conj |
ketoacidosis,levels |
R5258 |
T20145 |
T20144 |
punct |
", ",ketoacidosis |
R5259 |
T20146 |
T20147 |
compound |
brain,edema |
R5260 |
T20147 |
T20144 |
conj |
edema,ketoacidosis |
R5261 |
T20148 |
T20147 |
punct |
", ",edema |
R5262 |
T20149 |
T20147 |
conj |
dehydration,edema |
R5263 |
T20150 |
T20149 |
punct |
", ",dehydration |
R5264 |
T20151 |
T20149 |
cc |
and,dehydration |
R5265 |
T20152 |
T20149 |
conj |
malnutrition,dehydration |
R5266 |
T20153 |
T20154 |
mark |
as,observed |
R5267 |
T20154 |
T20132 |
advcl |
observed,is |
R5268 |
T20155 |
T20154 |
prep |
in,observed |
R5269 |
T20156 |
T20157 |
det |
the,calf |
R5270 |
T20157 |
T20155 |
pobj |
calf,in |
R5271 |
T20158 |
T20157 |
compound |
MSUD,calf |
R5272 |
T20159 |
T20160 |
punct |
[,17 |
R5273 |
T20160 |
T20157 |
parataxis |
17,calf |
R5274 |
T20161 |
T20160 |
punct |
],17 |
R5275 |
T20162 |
T20155 |
cc |
and,in |
R5276 |
T20163 |
T20155 |
conj |
in,in |
R5277 |
T20164 |
T20165 |
amod |
classic,patients |
R5278 |
T20165 |
T20163 |
pobj |
patients,in |
R5279 |
T20166 |
T20165 |
compound |
MSUD,patients |
R5280 |
T20167 |
T20168 |
punct |
[,1 |
R5281 |
T20168 |
T20165 |
parataxis |
1,patients |
R5282 |
T20169 |
T20168 |
punct |
],1 |
R5283 |
T20170 |
T20132 |
punct |
.,is |
R5284 |
T20172 |
T20173 |
amod |
Heterozygous,knockouts |
R5285 |
T20173 |
T20174 |
nsubj |
knockouts,were |
R5286 |
T20175 |
T20174 |
acomp |
normal,were |
R5287 |
T20176 |
T20174 |
cc |
and,were |
R5288 |
T20177 |
T20174 |
conj |
had,were |
R5289 |
T20178 |
T20179 |
amod |
normal,levels |
R5290 |
T20179 |
T20177 |
dobj |
levels,had |
R5291 |
T20180 |
T20179 |
prep |
of,levels |
R5292 |
T20181 |
T20180 |
pobj |
BCAA,of |
R5293 |
T20182 |
T20177 |
prep |
despite,had |
R5294 |
T20183 |
T20182 |
pcomp |
having,despite |
R5295 |
T20184 |
T20185 |
advmod |
approximately,half |
R5296 |
T20185 |
T20183 |
dobj |
half,having |
R5297 |
T20186 |
T20185 |
prep |
of,half |
R5298 |
T20187 |
T20188 |
nmod |
BCKDH,activity |
R5299 |
T20188 |
T20186 |
pobj |
activity,of |
R5300 |
T20189 |
T20188 |
amod |
enzymatic,activity |
R5301 |
T20190 |
T20174 |
punct |
.,were |
R5302 |
T20192 |
T20193 |
prep |
From,represents |
R5303 |
T20194 |
T20195 |
det |
the,studies |
R5304 |
T20195 |
T20192 |
pobj |
studies,From |
R5305 |
T20196 |
T20195 |
compound |
characterization,studies |
R5306 |
T20197 |
T20195 |
acl |
completed,studies |
R5307 |
T20198 |
T20199 |
advmod |
thus,far |
R5308 |
T20199 |
T20197 |
advmod |
far,completed |
R5309 |
T20200 |
T20193 |
punct |
", ",represents |
R5310 |
T20201 |
T20202 |
det |
the,mouse |
R5311 |
T20202 |
T20193 |
nsubj |
mouse,represents |
R5312 |
T20203 |
T20204 |
amod |
null,mutation |
R5313 |
T20204 |
T20202 |
compound |
mutation,mouse |
R5314 |
T20205 |
T20193 |
advmod |
accurately,represents |
R5315 |
T20206 |
T20207 |
det |
a,model |
R5316 |
T20207 |
T20193 |
dobj |
model,represents |
R5317 |
T20208 |
T20207 |
prep |
of,model |
R5318 |
T20209 |
T20210 |
amod |
classic,MSUD |
R5319 |
T20210 |
T20208 |
pobj |
MSUD,of |
R5320 |
T20211 |
T20193 |
cc |
and,represents |
R5321 |
T20212 |
T20193 |
conj |
appears,represents |
R5322 |
T20213 |
T20214 |
aux |
to,be |
R5323 |
T20214 |
T20212 |
xcomp |
be,appears |
R5324 |
T20215 |
T20216 |
det |
a,model |
R5325 |
T20216 |
T20214 |
attr |
model,be |
R5326 |
T20217 |
T20216 |
amod |
faithful,model |
R5327 |
T20218 |
T20216 |
prep |
of,model |
R5328 |
T20219 |
T20220 |
det |
the,disease |
R5329 |
T20220 |
T20218 |
pobj |
disease,of |
R5330 |
T20221 |
T20220 |
amod |
human,disease |
R5331 |
T20222 |
T20214 |
prep |
with,be |
R5332 |
T20223 |
T20222 |
pobj |
respect,with |
R5333 |
T20224 |
T20223 |
prep |
to,respect |
R5334 |
T20225 |
T20226 |
amod |
several,phenotypes |
R5335 |
T20226 |
T20224 |
pobj |
phenotypes,to |
R5336 |
T20227 |
T20226 |
amod |
biochemical,phenotypes |
R5337 |
T20228 |
T20229 |
punct |
[,1 |
R5338 |
T20229 |
T20212 |
parataxis |
1,appears |
R5339 |
T20230 |
T20229 |
punct |
],1 |
R5340 |
T20231 |
T20193 |
punct |
.,represents |