Id |
Subject |
Object |
Predicate |
Lexical cue |
T696 |
11-19 |
JJ |
denotes |
Juvenile |
T697 |
36-50 |
NN |
denotes |
lipofuscinosis |
T698 |
20-28 |
JJ |
denotes |
neuronal |
T699 |
29-35 |
NN |
denotes |
ceroid |
T700 |
78-80 |
VBZ |
denotes |
is |
T701 |
51-52 |
-LRB- |
denotes |
( |
T702 |
52-56 |
NN |
denotes |
JNCL |
T703 |
56-57 |
-RRB- |
denotes |
) |
T704 |
57-59 |
, |
denotes |
, |
T705 |
59-61 |
CC |
denotes |
or |
T706 |
62-68 |
NNP |
denotes |
Batten |
T707 |
69-76 |
NN |
denotes |
disease |
T708 |
76-78 |
, |
denotes |
, |
T709 |
81-82 |
DT |
denotes |
a |
T710 |
139-147 |
NN |
denotes |
disorder |
T711 |
83-94 |
RB |
denotes |
recessively |
T712 |
95-104 |
VBN |
denotes |
inherited |
T713 |
105-114 |
NN |
denotes |
childhood |
T714 |
114-115 |
HYPH |
denotes |
- |
T715 |
115-120 |
JJ |
denotes |
onset |
T716 |
121-138 |
JJ |
denotes |
neurodegenerative |
T717 |
148-161 |
VBN |
denotes |
characterized |
T718 |
162-164 |
IN |
denotes |
by |
T719 |
165-176 |
JJ |
denotes |
progressive |
T720 |
177-186 |
NN |
denotes |
blindness |
T721 |
186-188 |
, |
denotes |
, |
T722 |
188-196 |
NNS |
denotes |
seizures |
T723 |
196-198 |
, |
denotes |
, |
T724 |
198-203 |
NN |
denotes |
motor |
T725 |
218-225 |
NN |
denotes |
decline |
T726 |
204-207 |
CC |
denotes |
and |
T727 |
208-217 |
JJ |
denotes |
cognitive |
T728 |
225-227 |
, |
denotes |
, |
T729 |
227-230 |
CC |
denotes |
and |
T730 |
231-236 |
JJ |
denotes |
early |
T731 |
237-242 |
NN |
denotes |
death |
T732 |
243-244 |
-LRB- |
denotes |
[ |
T733 |
244-245 |
CD |
denotes |
1 |
T734 |
245-246 |
-RRB- |
denotes |
] |
T735 |
246-247 |
. |
denotes |
. |
T736 |
247-479 |
sentence |
denotes |
The primary genetic defect (>80% disease chromosomes) leading to JNCL is a 1.02 kb genomic DNA deletion in the CLN3 gene, which eliminates exons 7 and 8 and surrounding intronic DNA, predicting a non-functional protein product [2]. |
T737 |
248-251 |
DT |
denotes |
The |
T738 |
268-274 |
NN |
denotes |
defect |
T739 |
252-259 |
JJ |
denotes |
primary |
T740 |
260-267 |
JJ |
denotes |
genetic |
T741 |
318-320 |
VBZ |
denotes |
is |
T742 |
275-276 |
-LRB- |
denotes |
( |
T743 |
289-300 |
NNS |
denotes |
chromosomes |
T744 |
276-277 |
SYM |
denotes |
> |
T745 |
277-279 |
CD |
denotes |
80 |
T746 |
279-280 |
NN |
denotes |
% |
T747 |
281-288 |
NN |
denotes |
disease |
T748 |
300-301 |
-RRB- |
denotes |
) |
T749 |
302-309 |
VBG |
denotes |
leading |
T750 |
310-312 |
IN |
denotes |
to |
T751 |
313-317 |
NN |
denotes |
JNCL |
T752 |
321-322 |
DT |
denotes |
a |
T753 |
343-351 |
NN |
denotes |
deletion |
T754 |
323-327 |
CD |
denotes |
1.02 |
T755 |
328-330 |
NN |
denotes |
kb |
T756 |
331-338 |
JJ |
denotes |
genomic |
T757 |
339-342 |
NN |
denotes |
DNA |
T758 |
352-354 |
IN |
denotes |
in |
T759 |
355-358 |
DT |
denotes |
the |
T760 |
364-368 |
NN |
denotes |
gene |
T761 |
359-363 |
NN |
denotes |
CLN3 |
T762 |
368-370 |
, |
denotes |
, |
T763 |
370-375 |
WDT |
denotes |
which |
T764 |
376-386 |
VBZ |
denotes |
eliminates |
T765 |
387-392 |
NNS |
denotes |
exons |
T766 |
393-394 |
CD |
denotes |
7 |
T767 |
395-398 |
CC |
denotes |
and |
T768 |
399-400 |
CD |
denotes |
8 |
T769 |
401-404 |
CC |
denotes |
and |
T770 |
405-416 |
JJ |
denotes |
surrounding |
T771 |
426-429 |
NN |
denotes |
DNA |
T772 |
417-425 |
JJ |
denotes |
intronic |
T773 |
429-431 |
, |
denotes |
, |
T774 |
431-441 |
VBG |
denotes |
predicting |
T775 |
442-443 |
DT |
denotes |
a |
T776 |
467-474 |
NN |
denotes |
product |
T777 |
444-458 |
JJ |
denotes |
non-functional |
T778 |
459-466 |
NN |
denotes |
protein |
T779 |
475-476 |
-LRB- |
denotes |
[ |
T780 |
476-477 |
CD |
denotes |
2 |
T781 |
477-478 |
-RRB- |
denotes |
] |
T782 |
478-479 |
. |
denotes |
. |
T783 |
479-660 |
sentence |
denotes |
The pathological hallmark of JNCL is autofluorescent ceroid lipofuscin deposits within autolysosomes that are enriched in subunit c of the mitochondrial ATP synthase complex [3-5]. |
T784 |
480-483 |
DT |
denotes |
The |
T785 |
497-505 |
NN |
denotes |
hallmark |
T786 |
484-496 |
JJ |
denotes |
pathological |
T787 |
514-516 |
VBZ |
denotes |
is |
T788 |
506-508 |
IN |
denotes |
of |
T789 |
509-513 |
NN |
denotes |
JNCL |
T790 |
517-532 |
JJ |
denotes |
autofluorescent |
T791 |
551-559 |
NNS |
denotes |
deposits |
T792 |
533-539 |
NN |
denotes |
ceroid |
T793 |
540-550 |
NN |
denotes |
lipofuscin |
T794 |
560-566 |
IN |
denotes |
within |
T795 |
567-580 |
NNS |
denotes |
autolysosomes |
T796 |
581-585 |
WDT |
denotes |
that |
T797 |
586-589 |
VBP |
denotes |
are |
T798 |
590-598 |
JJ |
denotes |
enriched |
T799 |
599-601 |
IN |
denotes |
in |
T800 |
602-609 |
NN |
denotes |
subunit |
T801 |
610-611 |
NN |
denotes |
c |
T802 |
612-614 |
IN |
denotes |
of |
T803 |
615-618 |
DT |
denotes |
the |
T804 |
646-653 |
NN |
denotes |
complex |
T805 |
619-632 |
JJ |
denotes |
mitochondrial |
T806 |
637-645 |
NN |
denotes |
synthase |
T807 |
633-636 |
NN |
denotes |
ATP |
T808 |
654-655 |
-LRB- |
denotes |
[ |
T809 |
655-656 |
CD |
denotes |
3 |
T810 |
656-657 |
SYM |
denotes |
- |
T811 |
657-658 |
CD |
denotes |
5 |
T812 |
658-659 |
-RRB- |
denotes |
] |
T813 |
659-660 |
. |
denotes |
. |
T814 |
660-796 |
sentence |
denotes |
Remarkably, these deposits are not only found in CNS neurons but are also abundant in non-neuronal cells outside of the nervous system. |
T815 |
661-671 |
RB |
denotes |
Remarkably |
T816 |
701-706 |
VBN |
denotes |
found |
T817 |
671-673 |
, |
denotes |
, |
T818 |
673-678 |
DT |
denotes |
these |
T819 |
679-687 |
NNS |
denotes |
deposits |
T820 |
688-691 |
VBP |
denotes |
are |
T821 |
692-695 |
RB |
denotes |
not |
T822 |
696-700 |
RB |
denotes |
only |
T823 |
707-709 |
IN |
denotes |
in |
T824 |
710-713 |
NN |
denotes |
CNS |
T825 |
714-721 |
NNS |
denotes |
neurons |
T826 |
722-725 |
CC |
denotes |
but |
T827 |
726-729 |
VBP |
denotes |
are |
T828 |
730-734 |
RB |
denotes |
also |
T829 |
735-743 |
JJ |
denotes |
abundant |
T830 |
744-746 |
IN |
denotes |
in |
T831 |
747-759 |
JJ |
denotes |
non-neuronal |
T832 |
760-765 |
NNS |
denotes |
cells |
T833 |
766-773 |
IN |
denotes |
outside |
T834 |
774-776 |
IN |
denotes |
of |
T835 |
777-780 |
DT |
denotes |
the |
T836 |
789-795 |
NN |
denotes |
system |
T837 |
781-788 |
JJ |
denotes |
nervous |
T838 |
795-796 |
. |
denotes |
. |
T839 |
796-960 |
sentence |
denotes |
The relationship of subunit c deposits to the JNCL disease process, and the underlying reason for the neuronal specificity of the disease remain poorly understood. |
T840 |
797-800 |
DT |
denotes |
The |
T841 |
801-813 |
NN |
denotes |
relationship |
T842 |
935-941 |
VBP |
denotes |
remain |
T843 |
814-816 |
IN |
denotes |
of |
T844 |
817-824 |
NN |
denotes |
subunit |
T845 |
825-826 |
NN |
denotes |
c |
T846 |
827-835 |
NNS |
denotes |
deposits |
T847 |
836-838 |
IN |
denotes |
to |
T848 |
839-842 |
DT |
denotes |
the |
T849 |
856-863 |
NN |
denotes |
process |
T850 |
843-847 |
NN |
denotes |
JNCL |
T851 |
848-855 |
NN |
denotes |
disease |
T852 |
863-865 |
, |
denotes |
, |
T853 |
865-868 |
CC |
denotes |
and |
T854 |
869-872 |
DT |
denotes |
the |
T855 |
884-890 |
NN |
denotes |
reason |
T856 |
873-883 |
JJ |
denotes |
underlying |
T857 |
891-894 |
IN |
denotes |
for |
T858 |
895-898 |
DT |
denotes |
the |
T859 |
908-919 |
NN |
denotes |
specificity |
T860 |
899-907 |
JJ |
denotes |
neuronal |
T861 |
920-922 |
IN |
denotes |
of |
T862 |
923-926 |
DT |
denotes |
the |
T863 |
927-934 |
NN |
denotes |
disease |
T864 |
942-948 |
RB |
denotes |
poorly |
T865 |
949-959 |
VBN |
denotes |
understood |
T866 |
959-960 |
. |
denotes |
. |
T867 |
960-1174 |
sentence |
denotes |
The CLN3-encoded protein (battenin, also called CLN3 or cln3 p) is a highly conserved, ubiquitously expressed, multi-pass membrane protein [6] that localizes to the lysosome and other vesicular compartments [7-9]. |
T868 |
961-964 |
DT |
denotes |
The |
T869 |
978-985 |
NN |
denotes |
protein |
T870 |
965-969 |
NN |
denotes |
CLN3 |
T871 |
970-977 |
VBN |
denotes |
encoded |
T872 |
969-970 |
HYPH |
denotes |
- |
T873 |
1025-1027 |
VBZ |
denotes |
is |
T874 |
986-987 |
-LRB- |
denotes |
( |
T875 |
987-995 |
NN |
denotes |
battenin |
T876 |
995-997 |
, |
denotes |
, |
T877 |
997-1001 |
RB |
denotes |
also |
T878 |
1002-1008 |
VBN |
denotes |
called |
T879 |
1009-1013 |
NN |
denotes |
CLN3 |
T880 |
1014-1016 |
CC |
denotes |
or |
T881 |
1017-1021 |
NN |
denotes |
cln3 |
T882 |
1022-1023 |
NN |
denotes |
p |
T883 |
1023-1024 |
-RRB- |
denotes |
) |
T884 |
1028-1029 |
DT |
denotes |
a |
T885 |
1092-1099 |
NN |
denotes |
protein |
T886 |
1030-1036 |
RB |
denotes |
highly |
T887 |
1037-1046 |
JJ |
denotes |
conserved |
T888 |
1046-1048 |
, |
denotes |
, |
T889 |
1048-1060 |
RB |
denotes |
ubiquitously |
T890 |
1061-1070 |
VBN |
denotes |
expressed |
T891 |
1070-1072 |
, |
denotes |
, |
T892 |
1072-1082 |
NN |
denotes |
multi-pass |
T893 |
1083-1091 |
NN |
denotes |
membrane |
T894 |
1100-1101 |
-LRB- |
denotes |
[ |
T895 |
1101-1102 |
CD |
denotes |
6 |
T896 |
1102-1103 |
-RRB- |
denotes |
] |
T897 |
1104-1108 |
WDT |
denotes |
that |
T898 |
1109-1118 |
VBZ |
denotes |
localizes |
T899 |
1119-1121 |
IN |
denotes |
to |
T900 |
1122-1125 |
DT |
denotes |
the |
T901 |
1126-1134 |
NN |
denotes |
lysosome |
T902 |
1135-1138 |
CC |
denotes |
and |
T903 |
1139-1144 |
JJ |
denotes |
other |
T904 |
1155-1167 |
NNS |
denotes |
compartments |
T905 |
1145-1154 |
JJ |
denotes |
vesicular |
T906 |
1168-1169 |
-LRB- |
denotes |
[ |
T907 |
1169-1170 |
CD |
denotes |
7 |
T908 |
1170-1171 |
SYM |
denotes |
- |
T909 |
1171-1172 |
CD |
denotes |
9 |
T910 |
1172-1173 |
-RRB- |
denotes |
] |
T911 |
1173-1174 |
. |
denotes |
. |
T912 |
1174-1356 |
sentence |
denotes |
Battenin function remains to be elucidated, although studies of btn1, the yeast CLN3 ortholog, have implicated battenin in lysosomal pH homeostasis and amino acid transport [10,11]. |
T913 |
1175-1183 |
NN |
denotes |
Battenin |
T914 |
1184-1192 |
NN |
denotes |
function |
T915 |
1193-1200 |
VBZ |
denotes |
remains |
T916 |
1201-1203 |
TO |
denotes |
to |
T917 |
1207-1217 |
VBN |
denotes |
elucidated |
T918 |
1204-1206 |
VB |
denotes |
be |
T919 |
1217-1219 |
, |
denotes |
, |
T920 |
1219-1227 |
IN |
denotes |
although |
T921 |
1275-1285 |
VBN |
denotes |
implicated |
T922 |
1228-1235 |
NNS |
denotes |
studies |
T923 |
1236-1238 |
IN |
denotes |
of |
T924 |
1239-1243 |
NN |
denotes |
btn1 |
T925 |
1243-1245 |
, |
denotes |
, |
T926 |
1245-1248 |
DT |
denotes |
the |
T927 |
1260-1268 |
NN |
denotes |
ortholog |
T928 |
1249-1254 |
NN |
denotes |
yeast |
T929 |
1255-1259 |
NN |
denotes |
CLN3 |
T930 |
1268-1270 |
, |
denotes |
, |
T931 |
1270-1274 |
VBP |
denotes |
have |
T932 |
1286-1294 |
NN |
denotes |
battenin |
T933 |
1295-1297 |
IN |
denotes |
in |
T934 |
1298-1307 |
JJ |
denotes |
lysosomal |
T935 |
1311-1322 |
NN |
denotes |
homeostasis |
T936 |
1308-1310 |
NN |
denotes |
pH |
T937 |
1323-1326 |
CC |
denotes |
and |
T938 |
1327-1332 |
NN |
denotes |
amino |
T939 |
1333-1337 |
NN |
denotes |
acid |
T940 |
1338-1347 |
NN |
denotes |
transport |
T941 |
1348-1349 |
-LRB- |
denotes |
[ |
T942 |
1352-1354 |
CD |
denotes |
11 |
T943 |
1349-1351 |
CD |
denotes |
10 |
T944 |
1351-1352 |
, |
denotes |
, |
T945 |
1354-1355 |
-RRB- |
denotes |
] |
T946 |
1355-1356 |
. |
denotes |
. |
T947 |
1356-1472 |
sentence |
denotes |
To explore JNCL pathogenesis and battenin function, we previously generated a genetically precise JNCL mouse model. |
T948 |
1357-1359 |
TO |
denotes |
To |
T949 |
1360-1367 |
VB |
denotes |
explore |
T950 |
1423-1432 |
VBD |
denotes |
generated |
T951 |
1368-1372 |
NN |
denotes |
JNCL |
T952 |
1373-1385 |
NN |
denotes |
pathogenesis |
T953 |
1386-1389 |
CC |
denotes |
and |
T954 |
1390-1398 |
NN |
denotes |
battenin |
T955 |
1399-1407 |
NN |
denotes |
function |
T956 |
1407-1409 |
, |
denotes |
, |
T957 |
1409-1411 |
PRP |
denotes |
we |
T958 |
1412-1422 |
RB |
denotes |
previously |
T959 |
1433-1434 |
DT |
denotes |
a |
T960 |
1466-1471 |
NN |
denotes |
model |
T961 |
1435-1446 |
RB |
denotes |
genetically |
T962 |
1447-1454 |
JJ |
denotes |
precise |
T963 |
1455-1459 |
NN |
denotes |
JNCL |
T964 |
1460-1465 |
NN |
denotes |
mouse |
T965 |
1471-1472 |
. |
denotes |
. |
T966 |
1472-1655 |
sentence |
denotes |
Cln3Δex7/8 knock-in mice harbor the ~1 kb common JNCL mutation and express a non-truncated mutant battenin isoform that is detectable with antibodies recognizing C-terminal epitopes. |
T967 |
1473-1481 |
NN |
denotes |
Cln3Δex7 |
T968 |
1493-1497 |
NNS |
denotes |
mice |
T969 |
1481-1482 |
HYPH |
denotes |
/ |
T970 |
1482-1483 |
CD |
denotes |
8 |
T971 |
1484-1489 |
VB |
denotes |
knock |
T972 |
1489-1490 |
HYPH |
denotes |
- |
T973 |
1490-1492 |
RP |
denotes |
in |
T974 |
1498-1504 |
VBP |
denotes |
harbor |
T975 |
1505-1508 |
DT |
denotes |
the |
T976 |
1527-1535 |
NN |
denotes |
mutation |
T977 |
1509-1510 |
SYM |
denotes |
~ |
T978 |
1510-1511 |
CD |
denotes |
1 |
T979 |
1512-1514 |
NN |
denotes |
kb |
T980 |
1515-1521 |
JJ |
denotes |
common |
T981 |
1522-1526 |
NN |
denotes |
JNCL |
T982 |
1536-1539 |
CC |
denotes |
and |
T983 |
1540-1547 |
VBP |
denotes |
express |
T984 |
1548-1549 |
DT |
denotes |
a |
T985 |
1580-1587 |
NN |
denotes |
isoform |
T986 |
1550-1563 |
JJ |
denotes |
non-truncated |
T987 |
1564-1570 |
NN |
denotes |
mutant |
T988 |
1571-1579 |
NN |
denotes |
battenin |
T989 |
1588-1592 |
WDT |
denotes |
that |
T990 |
1593-1595 |
VBZ |
denotes |
is |
T991 |
1596-1606 |
JJ |
denotes |
detectable |
T992 |
1607-1611 |
IN |
denotes |
with |
T993 |
1612-1622 |
NNS |
denotes |
antibodies |
T994 |
1623-1634 |
VBG |
denotes |
recognizing |
T995 |
1635-1636 |
NN |
denotes |
C |
T996 |
1637-1645 |
JJ |
denotes |
terminal |
T997 |
1636-1637 |
HYPH |
denotes |
- |
T998 |
1646-1654 |
NNS |
denotes |
epitopes |
T999 |
1654-1655 |
. |
denotes |
. |
T1000 |
1655-1866 |
sentence |
denotes |
Homozygous Cln3Δex7/8 knock-in mice exhibit a progressive JNCL-like disease, with perinatal onset of subunit c deposition in many cell types and later onset of neuronal dysfunction and behavioral deficits [12]. |
T1001 |
1656-1666 |
JJ |
denotes |
Homozygous |
T1002 |
1687-1691 |
NNS |
denotes |
mice |
T1003 |
1667-1675 |
NN |
denotes |
Cln3Δex7 |
T1004 |
1675-1676 |
HYPH |
denotes |
/ |
T1005 |
1676-1677 |
CD |
denotes |
8 |
T1006 |
1678-1683 |
VB |
denotes |
knock |
T1007 |
1683-1684 |
HYPH |
denotes |
- |
T1008 |
1684-1686 |
RP |
denotes |
in |
T1009 |
1692-1699 |
VBP |
denotes |
exhibit |
T1010 |
1700-1701 |
DT |
denotes |
a |
T1011 |
1724-1731 |
NN |
denotes |
disease |
T1012 |
1702-1713 |
JJ |
denotes |
progressive |
T1013 |
1714-1718 |
NN |
denotes |
JNCL |
T1014 |
1719-1723 |
JJ |
denotes |
like |
T1015 |
1718-1719 |
HYPH |
denotes |
- |
T1016 |
1731-1733 |
, |
denotes |
, |
T1017 |
1733-1737 |
IN |
denotes |
with |
T1018 |
1738-1747 |
JJ |
denotes |
perinatal |
T1019 |
1748-1753 |
NN |
denotes |
onset |
T1020 |
1754-1756 |
IN |
denotes |
of |
T1021 |
1757-1764 |
NN |
denotes |
subunit |
T1022 |
1765-1766 |
NN |
denotes |
c |
T1023 |
1767-1777 |
NN |
denotes |
deposition |
T1024 |
1778-1780 |
IN |
denotes |
in |
T1025 |
1781-1785 |
JJ |
denotes |
many |
T1026 |
1791-1796 |
NNS |
denotes |
types |
T1027 |
1786-1790 |
NN |
denotes |
cell |
T1028 |
1797-1800 |
CC |
denotes |
and |
T1029 |
1801-1806 |
JJ |
denotes |
later |
T1030 |
1807-1812 |
NN |
denotes |
onset |
T1031 |
1813-1815 |
IN |
denotes |
of |
T1032 |
1816-1824 |
JJ |
denotes |
neuronal |
T1033 |
1825-1836 |
NN |
denotes |
dysfunction |
T1034 |
1837-1840 |
CC |
denotes |
and |
T1035 |
1841-1851 |
JJ |
denotes |
behavioral |
T1061 |
1977-1983 |
NN |
denotes |
manner |
T1036 |
1852-1860 |
NNS |
denotes |
deficits |
T1037 |
1861-1862 |
-LRB- |
denotes |
[ |
T1038 |
1862-1864 |
CD |
denotes |
12 |
T1039 |
1864-1865 |
-RRB- |
denotes |
] |
T1040 |
1865-1866 |
. |
denotes |
. |
T1041 |
1866-2025 |
sentence |
denotes |
These findings suggest that the major JNCL defect leads to abnormal turnover of mitochondrial subunit c, in a manner that selectively compromises CNS neurons. |
T1042 |
1867-1872 |
DT |
denotes |
These |
T1043 |
1873-1881 |
NNS |
denotes |
findings |
T1044 |
1882-1889 |
VBP |
denotes |
suggest |
T1045 |
1890-1894 |
IN |
denotes |
that |
T1046 |
1917-1922 |
VBZ |
denotes |
leads |
T1047 |
1895-1898 |
DT |
denotes |
the |
T1048 |
1910-1916 |
NN |
denotes |
defect |
T1049 |
1899-1904 |
JJ |
denotes |
major |
T1050 |
1905-1909 |
NN |
denotes |
JNCL |
T1051 |
1923-1925 |
IN |
denotes |
to |
T1052 |
1926-1934 |
JJ |
denotes |
abnormal |
T1053 |
1935-1943 |
NN |
denotes |
turnover |
T1054 |
1944-1946 |
IN |
denotes |
of |
T1055 |
1947-1960 |
JJ |
denotes |
mitochondrial |
T1056 |
1969-1970 |
NN |
denotes |
c |
T1057 |
1961-1968 |
NN |
denotes |
subunit |
T1058 |
1970-1972 |
, |
denotes |
, |
T1059 |
1972-1974 |
IN |
denotes |
in |
T1060 |
1975-1976 |
DT |
denotes |
a |
T1062 |
1984-1988 |
WDT |
denotes |
that |
T1063 |
2001-2012 |
VBZ |
denotes |
compromises |
T1064 |
1989-2000 |
RB |
denotes |
selectively |
T1065 |
2013-2016 |
NN |
denotes |
CNS |
T1066 |
2017-2024 |
NNS |
denotes |
neurons |
T1067 |
2024-2025 |
. |
denotes |
. |
T1068 |
2025-2157 |
sentence |
denotes |
Currently, there is no suitable neuronal cell system to investigate the impact of the common JNCL mutation on biological processes. |
T1069 |
2026-2035 |
RB |
denotes |
Currently |
T1070 |
2043-2045 |
VBZ |
denotes |
is |
T1071 |
2035-2037 |
, |
denotes |
, |
T1072 |
2037-2042 |
EX |
denotes |
there |
T1073 |
2046-2048 |
DT |
denotes |
no |
T1074 |
2072-2078 |
NN |
denotes |
system |
T1075 |
2049-2057 |
JJ |
denotes |
suitable |
T1076 |
2058-2066 |
JJ |
denotes |
neuronal |
T1077 |
2067-2071 |
NN |
denotes |
cell |
T1078 |
2079-2081 |
TO |
denotes |
to |
T1079 |
2082-2093 |
VB |
denotes |
investigate |
T1080 |
2094-2097 |
DT |
denotes |
the |
T1081 |
2098-2104 |
NN |
denotes |
impact |
T1082 |
2105-2107 |
IN |
denotes |
of |
T1083 |
2108-2111 |
DT |
denotes |
the |
T1084 |
2124-2132 |
NN |
denotes |
mutation |
T1085 |
2112-2118 |
JJ |
denotes |
common |
T1086 |
2119-2123 |
NN |
denotes |
JNCL |
T1087 |
2133-2135 |
IN |
denotes |
on |
T1088 |
2136-2146 |
JJ |
denotes |
biological |
T1089 |
2147-2156 |
NNS |
denotes |
processes |
T1090 |
2156-2157 |
. |
denotes |
. |
T1091 |
2157-2260 |
sentence |
denotes |
Therefore, we have established cerebellar neuronal precursor cell lines from Cln3Δex7/8 knock-in mice. |
T1092 |
2158-2167 |
RB |
denotes |
Therefore |
T1093 |
2177-2188 |
VBN |
denotes |
established |
T1094 |
2167-2169 |
, |
denotes |
, |
T1095 |
2169-2171 |
PRP |
denotes |
we |
T1096 |
2172-2176 |
VBP |
denotes |
have |
T1097 |
2189-2199 |
JJ |
denotes |
cerebellar |
T1098 |
2224-2229 |
NNS |
denotes |
lines |
T1099 |
2200-2208 |
JJ |
denotes |
neuronal |
T1100 |
2209-2218 |
NN |
denotes |
precursor |
T1101 |
2219-2223 |
NN |
denotes |
cell |
T1102 |
2230-2234 |
IN |
denotes |
from |
T1103 |
2235-2243 |
NN |
denotes |
Cln3Δex7 |
T1104 |
2255-2259 |
NNS |
denotes |
mice |
T1105 |
2243-2244 |
HYPH |
denotes |
/ |
T1106 |
2244-2245 |
CD |
denotes |
8 |
T1107 |
2246-2251 |
VB |
denotes |
knock |
T1108 |
2251-2252 |
HYPH |
denotes |
- |
T1109 |
2252-2254 |
RP |
denotes |
in |
T1110 |
2259-2260 |
. |
denotes |
. |
T1111 |
2260-2482 |
sentence |
denotes |
Homozygous CbCln3Δex7/8 cells exhibit pathological hallmarks of the disease, and a survey of membrane organelles revealed membrane trafficking defects and mitochondrial dysfunction in homozygous mutant CbCln3Δex7/8 cells. |
T1112 |
2261-2271 |
JJ |
denotes |
Homozygous |
T1113 |
2285-2290 |
NNS |
denotes |
cells |
T1114 |
2272-2282 |
NN |
denotes |
CbCln3Δex7 |
T1115 |
2282-2283 |
HYPH |
denotes |
/ |
T1116 |
2283-2284 |
CD |
denotes |
8 |
T1117 |
2291-2298 |
VBP |
denotes |
exhibit |
T1118 |
2299-2311 |
JJ |
denotes |
pathological |
T1119 |
2312-2321 |
NNS |
denotes |
hallmarks |
T1120 |
2322-2324 |
IN |
denotes |
of |
T1121 |
2325-2328 |
DT |
denotes |
the |
T1122 |
2329-2336 |
NN |
denotes |
disease |
T1123 |
2336-2338 |
, |
denotes |
, |
T1124 |
2338-2341 |
CC |
denotes |
and |
T1125 |
2342-2343 |
DT |
denotes |
a |
T1126 |
2344-2350 |
NN |
denotes |
survey |
T1127 |
2374-2382 |
VBD |
denotes |
revealed |
T1128 |
2351-2353 |
IN |
denotes |
of |
T1129 |
2354-2362 |
NN |
denotes |
membrane |
T1130 |
2363-2373 |
NNS |
denotes |
organelles |
T1131 |
2383-2391 |
NN |
denotes |
membrane |
T1132 |
2392-2403 |
VBG |
denotes |
trafficking |
T1133 |
2404-2411 |
NNS |
denotes |
defects |
T1134 |
2412-2415 |
CC |
denotes |
and |
T1135 |
2416-2429 |
JJ |
denotes |
mitochondrial |
T1136 |
2430-2441 |
NN |
denotes |
dysfunction |
T1137 |
2442-2444 |
IN |
denotes |
in |
T1138 |
2445-2455 |
JJ |
denotes |
homozygous |
T1139 |
2456-2462 |
NN |
denotes |
mutant |
T1140 |
2476-2481 |
NNS |
denotes |
cells |
T1141 |
2463-2473 |
NN |
denotes |
CbCln3Δex7 |
T1142 |
2473-2474 |
HYPH |
denotes |
/ |
T1143 |
2474-2475 |
CD |
denotes |
8 |
T1144 |
2481-2482 |
. |
denotes |
. |
R266 |
T696 |
T697 |
amod |
Juvenile,lipofuscinosis |
R267 |
T697 |
T700 |
nsubj |
lipofuscinosis,is |
R268 |
T698 |
T697 |
amod |
neuronal,lipofuscinosis |
R269 |
T699 |
T697 |
compound |
ceroid,lipofuscinosis |
R270 |
T701 |
T697 |
punct |
(,lipofuscinosis |
R271 |
T702 |
T697 |
appos |
JNCL,lipofuscinosis |
R272 |
T703 |
T697 |
punct |
),lipofuscinosis |
R273 |
T704 |
T697 |
punct |
", ",lipofuscinosis |
R274 |
T705 |
T697 |
cc |
or,lipofuscinosis |
R275 |
T706 |
T707 |
compound |
Batten,disease |
R276 |
T707 |
T697 |
conj |
disease,lipofuscinosis |
R277 |
T708 |
T700 |
punct |
", ",is |
R278 |
T709 |
T710 |
det |
a,disorder |
R279 |
T710 |
T700 |
attr |
disorder,is |
R280 |
T711 |
T712 |
advmod |
recessively,inherited |
R281 |
T712 |
T710 |
amod |
inherited,disorder |
R282 |
T713 |
T710 |
nmod |
childhood,disorder |
R283 |
T714 |
T713 |
punct |
-,childhood |
R284 |
T715 |
T713 |
amod |
onset,childhood |
R285 |
T716 |
T710 |
amod |
neurodegenerative,disorder |
R286 |
T717 |
T710 |
acl |
characterized,disorder |
R287 |
T718 |
T717 |
agent |
by,characterized |
R288 |
T719 |
T720 |
amod |
progressive,blindness |
R289 |
T720 |
T718 |
pobj |
blindness,by |
R290 |
T721 |
T720 |
punct |
", ",blindness |
R291 |
T722 |
T720 |
conj |
seizures,blindness |
R292 |
T723 |
T722 |
punct |
", ",seizures |
R293 |
T724 |
T725 |
nmod |
motor,decline |
R294 |
T725 |
T722 |
conj |
decline,seizures |
R295 |
T726 |
T724 |
cc |
and,motor |
R296 |
T727 |
T724 |
conj |
cognitive,motor |
R297 |
T728 |
T725 |
punct |
", ",decline |
R298 |
T729 |
T725 |
cc |
and,decline |
R299 |
T730 |
T731 |
amod |
early,death |
R300 |
T731 |
T725 |
conj |
death,decline |
R301 |
T732 |
T733 |
punct |
[,1 |
R302 |
T733 |
T717 |
parataxis |
1,characterized |
R303 |
T734 |
T733 |
punct |
],1 |
R304 |
T735 |
T700 |
punct |
.,is |
R305 |
T737 |
T738 |
det |
The,defect |
R306 |
T738 |
T741 |
nsubj |
defect,is |
R307 |
T739 |
T738 |
amod |
primary,defect |
R308 |
T740 |
T738 |
amod |
genetic,defect |
R309 |
T742 |
T743 |
punct |
(,chromosomes |
R311 |
T744 |
T745 |
punct |
>,80 |
R312 |
T745 |
T746 |
nummod |
80,% |
R313 |
T746 |
T743 |
compound |
%,chromosomes |
R314 |
T747 |
T743 |
compound |
disease,chromosomes |
R315 |
T748 |
T743 |
punct |
),chromosomes |
R316 |
T749 |
T738 |
acl |
leading,defect |
R317 |
T750 |
T749 |
prep |
to,leading |
R318 |
T751 |
T750 |
pobj |
JNCL,to |
R319 |
T752 |
T753 |
det |
a,deletion |
R321 |
T754 |
T755 |
nummod |
1.02,kb |
R322 |
T755 |
T753 |
nmod |
kb,deletion |
R323 |
T756 |
T753 |
amod |
genomic,deletion |
R324 |
T757 |
T753 |
compound |
DNA,deletion |
R325 |
T758 |
T753 |
prep |
in,deletion |
R326 |
T759 |
T760 |
det |
the,gene |
R327 |
T760 |
T758 |
pobj |
gene,in |
R328 |
T761 |
T760 |
compound |
CLN3,gene |
R329 |
T762 |
T753 |
punct |
", ",deletion |
R330 |
T763 |
T764 |
dep |
which,eliminates |
R331 |
T764 |
T753 |
relcl |
eliminates,deletion |
R332 |
T765 |
T766 |
nmod |
exons,7 |
R333 |
T766 |
T764 |
dobj |
7,eliminates |
R334 |
T767 |
T766 |
cc |
and,7 |
R335 |
T768 |
T766 |
conj |
8,7 |
R336 |
T769 |
T766 |
cc |
and,7 |
R337 |
T770 |
T771 |
amod |
surrounding,DNA |
R338 |
T771 |
T766 |
conj |
DNA,7 |
R339 |
T772 |
T771 |
amod |
intronic,DNA |
R340 |
T773 |
T741 |
punct |
", ",is |
R341 |
T774 |
T741 |
advcl |
predicting,is |
R342 |
T775 |
T776 |
det |
a,product |
R343 |
T776 |
T774 |
dobj |
product,predicting |
R344 |
T777 |
T776 |
amod |
non-functional,product |
R345 |
T778 |
T776 |
compound |
protein,product |
R346 |
T779 |
T780 |
punct |
[,2 |
R347 |
T780 |
T741 |
parataxis |
2,is |
R348 |
T781 |
T780 |
punct |
],2 |
R349 |
T782 |
T741 |
punct |
.,is |
R350 |
T784 |
T785 |
det |
The,hallmark |
R351 |
T785 |
T787 |
nsubj |
hallmark,is |
R352 |
T786 |
T785 |
amod |
pathological,hallmark |
R353 |
T788 |
T785 |
prep |
of,hallmark |
R354 |
T789 |
T788 |
pobj |
JNCL,of |
R355 |
T790 |
T791 |
amod |
autofluorescent,deposits |
R356 |
T791 |
T787 |
attr |
deposits,is |
R357 |
T792 |
T791 |
compound |
ceroid,deposits |
R358 |
T793 |
T791 |
compound |
lipofuscin,deposits |
R359 |
T794 |
T791 |
prep |
within,deposits |
R360 |
T795 |
T794 |
pobj |
autolysosomes,within |
R361 |
T796 |
T797 |
dep |
that,are |
R362 |
T797 |
T791 |
relcl |
are,deposits |
R363 |
T798 |
T797 |
acomp |
enriched,are |
R364 |
T799 |
T797 |
prep |
in,are |
R365 |
T800 |
T801 |
compound |
subunit,c |
R366 |
T801 |
T799 |
pobj |
c,in |
R367 |
T802 |
T801 |
prep |
of,c |
R368 |
T803 |
T804 |
det |
the,complex |
R369 |
T804 |
T802 |
pobj |
complex,of |
R370 |
T805 |
T806 |
amod |
mitochondrial,synthase |
R371 |
T806 |
T804 |
compound |
synthase,complex |
R372 |
T807 |
T806 |
compound |
ATP,synthase |
R373 |
T808 |
T809 |
punct |
[,3 |
R374 |
T809 |
T787 |
parataxis |
3,is |
R375 |
T810 |
T811 |
punct |
-,5 |
R376 |
T811 |
T809 |
prep |
5,3 |
R377 |
T812 |
T809 |
punct |
],3 |
R378 |
T813 |
T787 |
punct |
.,is |
R379 |
T815 |
T816 |
advmod |
Remarkably,found |
R380 |
T817 |
T816 |
punct |
", ",found |
R381 |
T818 |
T819 |
det |
these,deposits |
R382 |
T819 |
T816 |
nsubjpass |
deposits,found |
R383 |
T820 |
T816 |
auxpass |
are,found |
R384 |
T821 |
T816 |
preconj |
not,found |
R385 |
T822 |
T821 |
advmod |
only,not |
R386 |
T823 |
T816 |
prep |
in,found |
R387 |
T824 |
T825 |
compound |
CNS,neurons |
R388 |
T825 |
T823 |
pobj |
neurons,in |
R389 |
T826 |
T816 |
cc |
but,found |
R390 |
T827 |
T816 |
conj |
are,found |
R391 |
T828 |
T827 |
advmod |
also,are |
R392 |
T829 |
T827 |
acomp |
abundant,are |
R393 |
T830 |
T827 |
prep |
in,are |
R394 |
T831 |
T832 |
amod |
non-neuronal,cells |
R395 |
T832 |
T830 |
pobj |
cells,in |
R396 |
T833 |
T832 |
prep |
outside,cells |
R397 |
T834 |
T833 |
prep |
of,outside |
R398 |
T835 |
T836 |
det |
the,system |
R399 |
T836 |
T834 |
pobj |
system,of |
R400 |
T837 |
T836 |
amod |
nervous,system |
R401 |
T838 |
T816 |
punct |
.,found |
R402 |
T840 |
T841 |
det |
The,relationship |
R403 |
T841 |
T842 |
nsubj |
relationship,remain |
R404 |
T843 |
T841 |
prep |
of,relationship |
R405 |
T844 |
T845 |
compound |
subunit,c |
R406 |
T845 |
T846 |
compound |
c,deposits |
R407 |
T846 |
T843 |
pobj |
deposits,of |
R408 |
T847 |
T841 |
prep |
to,relationship |
R409 |
T848 |
T849 |
det |
the,process |
R410 |
T849 |
T847 |
pobj |
process,to |
R411 |
T850 |
T851 |
compound |
JNCL,disease |
R412 |
T851 |
T849 |
compound |
disease,process |
R413 |
T852 |
T841 |
punct |
", ",relationship |
R414 |
T853 |
T841 |
cc |
and,relationship |
R415 |
T854 |
T855 |
det |
the,reason |
R416 |
T855 |
T841 |
conj |
reason,relationship |
R417 |
T856 |
T855 |
amod |
underlying,reason |
R418 |
T857 |
T855 |
prep |
for,reason |
R419 |
T858 |
T859 |
det |
the,specificity |
R420 |
T859 |
T857 |
pobj |
specificity,for |
R421 |
T860 |
T859 |
amod |
neuronal,specificity |
R422 |
T861 |
T859 |
prep |
of,specificity |
R423 |
T862 |
T863 |
det |
the,disease |
R424 |
T863 |
T861 |
pobj |
disease,of |
R425 |
T864 |
T865 |
advmod |
poorly,understood |
R426 |
T865 |
T842 |
acomp |
understood,remain |
R427 |
T866 |
T842 |
punct |
.,remain |
R428 |
T868 |
T869 |
det |
The,protein |
R429 |
T869 |
T873 |
nsubj |
protein,is |
R430 |
T870 |
T871 |
npadvmod |
CLN3,encoded |
R431 |
T871 |
T869 |
amod |
encoded,protein |
R432 |
T872 |
T871 |
punct |
-,encoded |
R433 |
T874 |
T869 |
punct |
(,protein |
R434 |
T875 |
T869 |
appos |
battenin,protein |
R435 |
T876 |
T875 |
punct |
", ",battenin |
R436 |
T877 |
T878 |
advmod |
also,called |
R437 |
T878 |
T875 |
acl |
called,battenin |
R438 |
T879 |
T878 |
oprd |
CLN3,called |
R439 |
T880 |
T879 |
cc |
or,CLN3 |
R440 |
T881 |
T882 |
compound |
cln3,p |
R441 |
T882 |
T879 |
conj |
p,CLN3 |
R442 |
T883 |
T873 |
punct |
),is |
R443 |
T884 |
T885 |
det |
a,protein |
R444 |
T885 |
T873 |
attr |
protein,is |
R445 |
T886 |
T887 |
advmod |
highly,conserved |
R446 |
T887 |
T885 |
amod |
conserved,protein |
R447 |
T888 |
T885 |
punct |
", ",protein |
R448 |
T889 |
T890 |
advmod |
ubiquitously,expressed |
R449 |
T890 |
T885 |
amod |
expressed,protein |
R450 |
T891 |
T885 |
punct |
", ",protein |
R451 |
T892 |
T885 |
compound |
multi-pass,protein |
R452 |
T893 |
T885 |
compound |
membrane,protein |
R453 |
T894 |
T895 |
punct |
[,6 |
R454 |
T895 |
T885 |
parataxis |
6,protein |
R455 |
T896 |
T895 |
punct |
],6 |
R456 |
T897 |
T898 |
dep |
that,localizes |
R457 |
T898 |
T885 |
relcl |
localizes,protein |
R458 |
T899 |
T898 |
prep |
to,localizes |
R459 |
T900 |
T901 |
det |
the,lysosome |
R460 |
T901 |
T899 |
pobj |
lysosome,to |
R461 |
T902 |
T901 |
cc |
and,lysosome |
R462 |
T903 |
T904 |
amod |
other,compartments |
R463 |
T904 |
T901 |
conj |
compartments,lysosome |
R464 |
T905 |
T904 |
amod |
vesicular,compartments |
R465 |
T906 |
T907 |
punct |
[,7 |
R466 |
T907 |
T898 |
parataxis |
7,localizes |
R467 |
T908 |
T909 |
punct |
-,9 |
R468 |
T909 |
T907 |
prep |
9,7 |
R469 |
T910 |
T907 |
punct |
],7 |
R470 |
T911 |
T873 |
punct |
.,is |
R471 |
T913 |
T914 |
compound |
Battenin,function |
R472 |
T914 |
T915 |
nsubj |
function,remains |
R473 |
T916 |
T917 |
aux |
to,elucidated |
R474 |
T917 |
T915 |
xcomp |
elucidated,remains |
R475 |
T918 |
T917 |
auxpass |
be,elucidated |
R476 |
T919 |
T917 |
punct |
", ",elucidated |
R477 |
T920 |
T921 |
mark |
although,implicated |
R478 |
T921 |
T917 |
advcl |
implicated,elucidated |
R479 |
T922 |
T921 |
nsubj |
studies,implicated |
R480 |
T923 |
T922 |
prep |
of,studies |
R481 |
T924 |
T923 |
pobj |
btn1,of |
R482 |
T925 |
T924 |
punct |
", ",btn1 |
R483 |
T926 |
T927 |
det |
the,ortholog |
R484 |
T927 |
T924 |
appos |
ortholog,btn1 |
R485 |
T928 |
T927 |
compound |
yeast,ortholog |
R486 |
T929 |
T927 |
compound |
CLN3,ortholog |
R487 |
T930 |
T921 |
punct |
", ",implicated |
R488 |
T931 |
T921 |
aux |
have,implicated |
R489 |
T932 |
T921 |
dobj |
battenin,implicated |
R490 |
T933 |
T921 |
prep |
in,implicated |
R491 |
T934 |
T935 |
amod |
lysosomal,homeostasis |
R492 |
T935 |
T933 |
pobj |
homeostasis,in |
R493 |
T936 |
T935 |
compound |
pH,homeostasis |
R494 |
T937 |
T935 |
cc |
and,homeostasis |
R495 |
T938 |
T939 |
compound |
amino,acid |
R496 |
T939 |
T940 |
compound |
acid,transport |
R497 |
T940 |
T935 |
conj |
transport,homeostasis |
R498 |
T941 |
T942 |
punct |
[,11 |
R499 |
T942 |
T921 |
parataxis |
11,implicated |
R500 |
T943 |
T942 |
nummod |
10,11 |
R501 |
T944 |
T942 |
punct |
",",11 |
R502 |
T945 |
T942 |
punct |
],11 |
R503 |
T946 |
T915 |
punct |
.,remains |
R504 |
T948 |
T949 |
aux |
To,explore |
R505 |
T949 |
T950 |
advcl |
explore,generated |
R506 |
T951 |
T952 |
compound |
JNCL,pathogenesis |
R507 |
T952 |
T949 |
dobj |
pathogenesis,explore |
R508 |
T953 |
T952 |
cc |
and,pathogenesis |
R509 |
T954 |
T955 |
compound |
battenin,function |
R510 |
T955 |
T952 |
conj |
function,pathogenesis |
R511 |
T956 |
T950 |
punct |
", ",generated |
R512 |
T957 |
T950 |
nsubj |
we,generated |
R513 |
T958 |
T950 |
advmod |
previously,generated |
R514 |
T959 |
T960 |
det |
a,model |
R516 |
T961 |
T962 |
advmod |
genetically,precise |
R517 |
T962 |
T960 |
amod |
precise,model |
R518 |
T963 |
T960 |
compound |
JNCL,model |
R519 |
T964 |
T960 |
compound |
mouse,model |
R520 |
T965 |
T950 |
punct |
.,generated |
R521 |
T967 |
T968 |
nmod |
Cln3Δex7,mice |
R522 |
T968 |
T974 |
nsubj |
mice,harbor |
R523 |
T969 |
T967 |
punct |
/,Cln3Δex7 |
R524 |
T970 |
T967 |
nummod |
8,Cln3Δex7 |
R525 |
T971 |
T968 |
amod |
knock,mice |
R526 |
T972 |
T971 |
punct |
-,knock |
R527 |
T973 |
T971 |
prt |
in,knock |
R528 |
T975 |
T976 |
det |
the,mutation |
R529 |
T976 |
T974 |
dobj |
mutation,harbor |
R530 |
T977 |
T978 |
punct |
~,1 |
R531 |
T978 |
T979 |
nummod |
1,kb |
R532 |
T979 |
T976 |
nmod |
kb,mutation |
R533 |
T980 |
T976 |
amod |
common,mutation |
R534 |
T981 |
T976 |
compound |
JNCL,mutation |
R535 |
T982 |
T974 |
cc |
and,harbor |
R536 |
T983 |
T974 |
conj |
express,harbor |
R537 |
T984 |
T985 |
det |
a,isoform |
R538 |
T985 |
T983 |
dobj |
isoform,express |
R539 |
T986 |
T985 |
amod |
non-truncated,isoform |
R540 |
T987 |
T988 |
compound |
mutant,battenin |
R541 |
T988 |
T985 |
compound |
battenin,isoform |
R542 |
T989 |
T990 |
dep |
that,is |
R543 |
T990 |
T985 |
relcl |
is,isoform |
R544 |
T991 |
T990 |
acomp |
detectable,is |
R545 |
T992 |
T990 |
prep |
with,is |
R546 |
T993 |
T992 |
pobj |
antibodies,with |
R547 |
T994 |
T993 |
acl |
recognizing,antibodies |
R548 |
T995 |
T996 |
npadvmod |
C,terminal |
R549 |
T996 |
T998 |
amod |
terminal,epitopes |
R550 |
T997 |
T996 |
punct |
-,terminal |
R551 |
T998 |
T994 |
dobj |
epitopes,recognizing |
R552 |
T999 |
T974 |
punct |
.,harbor |
R553 |
T1001 |
T1002 |
amod |
Homozygous,mice |
R554 |
T1002 |
T1009 |
nsubj |
mice,exhibit |
R555 |
T1003 |
T1002 |
nmod |
Cln3Δex7,mice |
R556 |
T1004 |
T1003 |
punct |
/,Cln3Δex7 |
R557 |
T1005 |
T1003 |
nummod |
8,Cln3Δex7 |
R558 |
T1006 |
T1002 |
amod |
knock,mice |
R559 |
T1007 |
T1006 |
punct |
-,knock |
R560 |
T1008 |
T1006 |
prt |
in,knock |
R561 |
T1010 |
T1011 |
det |
a,disease |
R563 |
T1012 |
T1011 |
amod |
progressive,disease |
R564 |
T1013 |
T1014 |
npadvmod |
JNCL,like |
R565 |
T1014 |
T1011 |
amod |
like,disease |
R566 |
T1015 |
T1014 |
punct |
-,like |
R567 |
T1016 |
T1011 |
punct |
", ",disease |
R568 |
T1017 |
T1011 |
prep |
with,disease |
R569 |
T1018 |
T1019 |
amod |
perinatal,onset |
R570 |
T1019 |
T1017 |
pobj |
onset,with |
R571 |
T1020 |
T1019 |
prep |
of,onset |
R572 |
T1021 |
T1022 |
compound |
subunit,c |
R573 |
T1022 |
T1023 |
compound |
c,deposition |
R574 |
T1023 |
T1020 |
pobj |
deposition,of |
R575 |
T1024 |
T1023 |
prep |
in,deposition |
R576 |
T1025 |
T1026 |
amod |
many,types |
R577 |
T1026 |
T1024 |
pobj |
types,in |
R578 |
T1027 |
T1026 |
compound |
cell,types |
R579 |
T1028 |
T1019 |
cc |
and,onset |
R580 |
T1029 |
T1030 |
amod |
later,onset |
R581 |
T1030 |
T1019 |
conj |
onset,onset |
R582 |
T1031 |
T1030 |
prep |
of,onset |
R583 |
T1032 |
T1033 |
amod |
neuronal,dysfunction |
R584 |
T1033 |
T1031 |
pobj |
dysfunction,of |
R585 |
T1034 |
T1033 |
cc |
and,dysfunction |
R586 |
T1035 |
T1036 |
amod |
behavioral,deficits |
R587 |
T1036 |
T1033 |
conj |
deficits,dysfunction |
R588 |
T1037 |
T1038 |
punct |
[,12 |
R589 |
T1038 |
T1009 |
parataxis |
12,exhibit |
R590 |
T1039 |
T1038 |
punct |
],12 |
R591 |
T1040 |
T1009 |
punct |
.,exhibit |
R592 |
T1042 |
T1043 |
det |
These,findings |
R593 |
T1043 |
T1044 |
nsubj |
findings,suggest |
R594 |
T1045 |
T1046 |
mark |
that,leads |
R596 |
T1047 |
T1048 |
det |
the,defect |
R597 |
T1048 |
T1046 |
nsubj |
defect,leads |
R598 |
T1049 |
T1048 |
amod |
major,defect |
R599 |
T1050 |
T1048 |
compound |
JNCL,defect |
R600 |
T1051 |
T1046 |
prep |
to,leads |
R601 |
T1052 |
T1053 |
amod |
abnormal,turnover |
R602 |
T1053 |
T1051 |
pobj |
turnover,to |
R603 |
T1054 |
T1053 |
prep |
of,turnover |
R604 |
T1055 |
T1056 |
amod |
mitochondrial,c |
R605 |
T1056 |
T1054 |
pobj |
c,of |
R606 |
T1057 |
T1056 |
compound |
subunit,c |
R607 |
T1058 |
T1046 |
punct |
", ",leads |
R608 |
T1059 |
T1046 |
prep |
in,leads |
R609 |
T1060 |
T1061 |
det |
a,manner |
R610 |
T1061 |
T1059 |
pobj |
manner,in |
R611 |
T1062 |
T1063 |
dep |
that,compromises |
R612 |
T1063 |
T1061 |
relcl |
compromises,manner |
R613 |
T1064 |
T1063 |
advmod |
selectively,compromises |
R614 |
T1065 |
T1066 |
compound |
CNS,neurons |
R615 |
T1066 |
T1063 |
dobj |
neurons,compromises |
R616 |
T1067 |
T1044 |
punct |
.,suggest |
R617 |
T1069 |
T1070 |
advmod |
Currently,is |
R618 |
T1071 |
T1070 |
punct |
", ",is |
R619 |
T1072 |
T1070 |
expl |
there,is |
R620 |
T1073 |
T1074 |
det |
no,system |
R621 |
T1074 |
T1070 |
attr |
system,is |
R622 |
T1075 |
T1074 |
amod |
suitable,system |
R623 |
T1076 |
T1077 |
amod |
neuronal,cell |
R624 |
T1077 |
T1074 |
compound |
cell,system |
R625 |
T1078 |
T1079 |
aux |
to,investigate |
R626 |
T1079 |
T1074 |
advcl |
investigate,system |
R627 |
T1080 |
T1081 |
det |
the,impact |
R628 |
T1081 |
T1079 |
dobj |
impact,investigate |
R629 |
T1082 |
T1081 |
prep |
of,impact |
R630 |
T1083 |
T1084 |
det |
the,mutation |
R631 |
T1084 |
T1082 |
pobj |
mutation,of |
R632 |
T1085 |
T1084 |
amod |
common,mutation |
R633 |
T1086 |
T1084 |
compound |
JNCL,mutation |
R634 |
T1087 |
T1081 |
prep |
on,impact |
R635 |
T1088 |
T1089 |
amod |
biological,processes |
R636 |
T1089 |
T1087 |
pobj |
processes,on |
R637 |
T1090 |
T1070 |
punct |
.,is |
R638 |
T1092 |
T1093 |
advmod |
Therefore,established |
R639 |
T1094 |
T1093 |
punct |
", ",established |
R640 |
T1095 |
T1093 |
nsubj |
we,established |
R641 |
T1096 |
T1093 |
aux |
have,established |
R642 |
T1097 |
T1098 |
amod |
cerebellar,lines |
R643 |
T1098 |
T1093 |
dobj |
lines,established |
R644 |
T1099 |
T1098 |
amod |
neuronal,lines |
R645 |
T1100 |
T1098 |
compound |
precursor,lines |
R646 |
T1101 |
T1098 |
compound |
cell,lines |
R647 |
T1102 |
T1093 |
prep |
from,established |
R648 |
T1103 |
T1104 |
nmod |
Cln3Δex7,mice |
R649 |
T1104 |
T1102 |
pobj |
mice,from |
R650 |
T1105 |
T1103 |
punct |
/,Cln3Δex7 |
R651 |
T1106 |
T1103 |
nummod |
8,Cln3Δex7 |
R652 |
T1107 |
T1104 |
amod |
knock,mice |
R653 |
T1108 |
T1107 |
punct |
-,knock |
R654 |
T1109 |
T1107 |
prt |
in,knock |
R655 |
T1110 |
T1093 |
punct |
.,established |
R656 |
T1112 |
T1113 |
amod |
Homozygous,cells |
R657 |
T1113 |
T1117 |
nsubj |
cells,exhibit |
R658 |
T1114 |
T1113 |
nmod |
CbCln3Δex7,cells |
R659 |
T1115 |
T1114 |
punct |
/,CbCln3Δex7 |
R660 |
T1116 |
T1114 |
nummod |
8,CbCln3Δex7 |
R661 |
T1118 |
T1119 |
amod |
pathological,hallmarks |
R662 |
T1119 |
T1117 |
dobj |
hallmarks,exhibit |
R663 |
T1120 |
T1119 |
prep |
of,hallmarks |
R664 |
T1121 |
T1122 |
det |
the,disease |
R665 |
T1122 |
T1120 |
pobj |
disease,of |
R666 |
T1123 |
T1117 |
punct |
", ",exhibit |
R667 |
T1124 |
T1117 |
cc |
and,exhibit |
R668 |
T1125 |
T1126 |
det |
a,survey |
R669 |
T1126 |
T1127 |
nsubj |
survey,revealed |
R670 |
T1127 |
T1117 |
conj |
revealed,exhibit |
R671 |
T1128 |
T1126 |
prep |
of,survey |
R672 |
T1129 |
T1130 |
compound |
membrane,organelles |
R673 |
T1130 |
T1128 |
pobj |
organelles,of |
R674 |
T1131 |
T1132 |
npadvmod |
membrane,trafficking |
R675 |
T1132 |
T1133 |
amod |
trafficking,defects |
R676 |
T1133 |
T1127 |
dobj |
defects,revealed |
R677 |
T1134 |
T1133 |
cc |
and,defects |
R678 |
T1135 |
T1136 |
amod |
mitochondrial,dysfunction |
R679 |
T1136 |
T1133 |
conj |
dysfunction,defects |
R680 |
T1137 |
T1127 |
prep |
in,revealed |
R681 |
T1138 |
T1139 |
amod |
homozygous,mutant |
R682 |
T1139 |
T1140 |
nmod |
mutant,cells |
R683 |
T1140 |
T1137 |
pobj |
cells,in |
R684 |
T1141 |
T1140 |
nmod |
CbCln3Δex7,cells |
R685 |
T1142 |
T1141 |
punct |
/,CbCln3Δex7 |
R686 |
T1143 |
T1141 |
nummod |
8,CbCln3Δex7 |
R687 |
T1144 |
T1127 |
punct |
.,revealed |
R310 |
T743 |
T738 |
parataxis |
chromosomes,defect |
R320 |
T753 |
T741 |
attr |
deletion,is |
R515 |
T960 |
T950 |
dobj |
model,generated |
R562 |
T1011 |
T1009 |
dobj |
disease,exhibit |
R595 |
T1046 |
T1044 |
ccomp |
leads,suggest |