242:
219:
116:
141:
500:
493:
507:
248:
147:
1966:
1169:. YME1L1 is a member of the AAA family of ATPases and has an important role for the maintenance of mitochondrial morphology. Its mature form assembles into a homo-oligomeric complex within the inner mitochondrial membrane (IM). It degrades both intermembrane space and IM proteins, including lipid transfer proteins, components of
1926:
Pellegrini L, Passer BJ, Canelles M, Lefterov I, Ganjei JK, Fowlkes BJ, Koonin EV, D'Adamio L (April 2001). "PAMP and PARL, two novel putative metalloproteases interacting with the COOH-terminus of
Presenilin-1 and -2".
1642:
Wai T, García-Prieto J, Baker MJ, Merkwirth C, Benit P, Rustin P, Rupérez FJ, Barbas C, Ibañez B, Langer T (December 2015). "Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice".
1225:
processing site and impairs YME1L1 maturation, leading to its rapid degradation, and also leads to a proliferation defect, abnormal OPA1 processing and mitochondrial fragmentation.
1883:
Zhang QH, Ye M, Wu XY, Ren SX, Zhao M, Zhao CJ, Fu G, Shen Y, Fan HY, Lu G, Zhong M, Xu XR, Han ZG, Zhang JW, Tao J, Huang QH, Zhou J, Hu GX, Gu J, Chen SJ, Chen Z (October 2000).
1354:
Hartmann B, Wai T, Hu H, MacVicar T, Musante L, Fischer-Zirnsak B, Stenzel W, Gräf R, van den Heuvel L, Ropers HH, Wienker TF, Hübner C, Langer T, Kaindl AM (6 August 2016).
255:
154:
1885:"Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells"
1408:
Coppola M, Pizzigoni A, Banfi S, Bassi MT, Casari G, Incerti B (May 2000). "Identification and characterization of YME1L1, a novel paraplegin-related gene".
765:
746:
77:
1747:"YME1L controls the accumulation of respiratory chain subunits and is required for apoptotic resistance, cristae morphogenesis, and cell proliferation"
1213:
mutation in the YME1L1 gene would cause infantile-onset mitochondriopathy, with severe intellectual disability, muscular impairments, and optic nerve
2035:
2011:
2030:
1315:
1297:
241:
1222:
989:
1170:
996:
218:
1333:
1284:
1263:
140:
115:
1280:
1259:
1155:
57:
254:
153:
2004:
247:
146:
1356:"Homozygous YME1L1 mutation causes mitochondriopathy with optic atrophy and mitochondrial network fragmentation"
810:
65:
1842:"The human homologue of the yeast mitochondrial AAA metalloprotease Yme1p complements a yeast yme1 disruptant"
1688:"Mitochondrial Phospholipid Homeostasis Is Regulated by the i-AAA Protease PaIAP and Affects Organismic Aging"
1127:
791:
1745:
Stiburek L, Cesnekova J, Kostkova O, Fornuskova D, Vinsova K, Wenchich L, Houstek J, Zeman J (March 2012).
1194:
1131:
1502:"TRIAP1/PRELI complexes prevent apoptosis by mediating intramitochondrial transport of phosphatidic acid"
1997:
1182:
129:
1185:, and mitochondrial network fragmentation. It's also reported that YME1L1 controls the accumulation of
44:
943:
884:
1445:"ATP-dependent proteases controlling mitochondrial function in the yeast Saccharomyces cerevisiae"
1871:
1668:
1482:
1198:
1135:
89:
972:
951:
947:
917:
896:
892:
888:
1158:
and is more abundant in tissues with a high content of mitochondria such as human adult heart,
499:
492:
1944:
1914:
1863:
1840:
Shah ZH, Hakkaart GA, Arku B, de Jong L, van der Spek H, Grivell LA, Jacobs HT (August 2000).
1817:
1776:
1727:
1709:
1660:
1624:
1575:
1523:
1474:
1425:
1387:
1218:
1186:
1075:
37:
1981:
1936:
1904:
1896:
1853:
1807:
1766:
1758:
1717:
1699:
1652:
1614:
1606:
1565:
1557:
1513:
1464:
1456:
1417:
1377:
1367:
506:
334:
265:
209:
164:
85:
1595:"The i-AAA protease YME1L and OMA1 cleave OPA1 to balance mitochondrial fusion and fission"
1123:
1159:
309:
1181:-dependent long-form OPA1 cleavage, resulting in short-form OPA1 accumulation, increased
1469:
1444:
1771:
1746:
1722:
1687:
1619:
1594:
1570:
1545:
1382:
1355:
1143:
1909:
1884:
1858:
1841:
1546:"YME1L degradation reduces mitochondrial proteolytic capacity during oxidative stress"
680:
675:
670:
665:
660:
655:
650:
645:
640:
624:
619:
614:
609:
604:
588:
583:
578:
573:
568:
563:
558:
553:
548:
2024:
1977:
1096:
535:
1875:
1672:
69:
1500:
Potting C, Tatsuta T, König T, Haag M, Wai T, Aaltonen MJ, Langer T (August 2013).
1486:
1063:
327:
106:
1686:
Löser, Timo; Joppe, Aljoscha; Hamann, Andrea; Osiewacz, Heinz D. (October 2021).
1593:
Anand R, Wai T, Baker MJ, Kladt N, Schauss AC, Rugarli E, Langer T (March 2014).
93:
1320:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1302:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1116:
1518:
1501:
410:
1210:
1119:
and is highly similar to all mitochondrial AAA proteases and in particular to
226:
123:
73:
1713:
1126:. Three different domains are identified via sequence analysis, including an
1762:
1656:
1561:
1190:
1071:
710:
470:
348:
293:
280:
192:
179:
81:
1948:
1918:
1867:
1821:
1780:
1731:
1664:
1628:
1579:
1527:
1478:
1429:
1421:
1391:
1940:
1704:
1610:
1460:
1036:
1031:
656:
protein quality control for misfolded or incompletely synthesized proteins
1163:
1104:
1020:
859:
840:
836:
1372:
1812:
1795:
1214:
1067:
822:
777:
1900:
1796:"OPA1 processing in cell death and disease - the long and short of it"
1070:
and mainly functions in the maintenance of mitochondrial morphology.
1052:
1004:
732:
1965:
1120:
1100:
695:
691:
1973:
1178:
1174:
1139:
1059:
515:
61:
1166:
317:
1985:
1544:
Rainbolt TK, Saunders JM, Wiseman RL (January 2015).
482:
48:, FTSH, MEG4, PAMP, YME1L, YME1 like 1 ATPase, OPA11
965:
936:
910:
877:
1276:
1274:
1272:
1255:
1253:
1251:
1334:"Entrez Gene: YME1L1 YME1-like 1 (S. cerevisiae)"
666:mitochondrial calcium ion transmembrane transport
264:
163:
1539:
1537:
1173:, and the dynamin-like GTPase optic atrophy 1 (
16:Protein-coding gene in the species Homo sapiens
1403:
1401:
1349:
1347:
1345:
1343:
1281:GRCm38: Ensembl release 89: ENSMUSG00000026775
2005:
1103:. Two transcript variants encoding different
8:
1260:GRCh38: Ensembl release 89: ENSG00000136758
2012:
1998:
706:
531:
305:
204:
101:
1908:
1857:
1811:
1770:
1721:
1703:
1618:
1569:
1517:
1468:
1381:
1371:
1074:in this gene would cause infantile-onset
671:negative regulation of apoptotic process
1247:
661:mitochondrial protein catabolic process
1443:Van Dyck L, Langer T (November 1999).
18:
269:
230:
225:
168:
127:
122:
7:
1962:
1960:
1449:Cellular and Molecular Life Sciences
1130:between amino acids 317 and 502, an
1049:ATP-dependent metalloprotease YME1L1
1984:. You can help Knowledge (XXG) by
1794:MacVicar T, Langer T (June 2016).
962:
933:
907:
874:
850:
827:
801:
782:
756:
737:
487:
405:
343:
322:
14:
1055:that in humans is encoded by the
1964:
681:mitochondrial protein processing
589:ATP-dependent peptidase activity
505:
498:
491:
253:
246:
240:
217:
152:
145:
139:
114:
1107:have been found for this gene.
2036:Human chromosome 10 gene stubs
1929:Journal of Alzheimer's Disease
1177:) Loss of YME1L1 accelerates
1171:protein translocases of the IM
605:integral component of membrane
516:More reference expression data
471:More reference expression data
1:
1859:10.1016/S0014-5793(00)01859-7
1751:Molecular Biology of the Cell
1189:subunits and is required for
651:cell population proliferation
564:metalloendopeptidase activity
238:
137:
2031:Genes on human chromosome 10
1156:inner mitochondrial membrane
625:mitochondrial inner membrane
387:Epithelium of choroid plexus
1599:The Journal of Cell Biology
2052:
1959:
1519:10.1016/j.cmet.2013.07.008
1154:YME1L1 is embedded in the
641:mitochondrion organization
1316:"Mouse PubMed Reference:"
1298:"Human PubMed Reference:"
1035:
1030:
1026:
1019:
1003:
984:
969:
940:
929:
914:
881:
870:
857:
853:
834:
830:
821:
808:
804:
789:
785:
776:
763:
759:
744:
740:
731:
716:
709:
705:
689:
549:metallopeptidase activity
534:
530:
513:
490:
481:
468:
417:
408:
355:
346:
316:
308:
304:
287:
274:
237:
216:
207:
203:
186:
173:
136:
113:
104:
100:
55:
52:
42:
35:
30:
26:
21:
1062:. YME1L1 belongs to the
990:Chr 10: 27.11 – 27.16 Mb
1800:Journal of Cell Science
1763:10.1091/mbc.E11-08-0674
1657:10.1126/science.aad0116
1562:10.15252/embr.201438976
1115:YME1L1 consists of 716
997:Chr 2: 23.05 – 23.09 Mb
1422:10.1006/geno.2000.6136
1128:AAA consensus sequence
676:protein hexamerization
1941:10.3233/jad-2001-3203
1705:10.3390/cells10102775
1611:10.1083/jcb.201308006
1461:10.1007/s000180050029
1205:Clinical significance
1195:cristae morphogenesis
1183:mitochondrial fission
1132:ATP/GTP binding motif
1099:14, consisting of 20
130:Chromosome 10 (human)
232:Chromosome 2 (mouse)
1373:10.7554/eLife.16078
1142:-dependent binding
1095:gene is located at
437:submandibular gland
429:seminiferous tubule
383:gingival epithelium
359:germinal epithelium
1972:This article on a
1813:10.1242/jcs.159186
1199:cell proliferation
811:ENSMUSG00000026775
634:Biological process
598:Cellular component
579:nucleotide binding
559:hydrolase activity
554:peptidase activity
542:Molecular function
391:tail of epididymis
94:YME1L1 - orthologs
1993:
1992:
1901:10.1101/gr.140200
1651:(6265): aad0116.
1219:missense mutation
1187:respiratory chain
1076:mitochondriopathy
1046:
1045:
1042:
1041:
1015:
1014:
980:
979:
959:
958:
925:
924:
904:
903:
866:
865:
847:
846:
817:
816:
798:
797:
772:
771:
753:
752:
701:
700:
574:metal ion binding
526:
525:
522:
521:
477:
476:
464:
463:
402:
401:
379:corpus epididymis
300:
299:
199:
198:
2043:
2014:
2007:
2000:
1968:
1961:
1952:
1922:
1912:
1879:
1861:
1826:
1825:
1815:
1806:(12): 2297–306.
1791:
1785:
1784:
1774:
1742:
1736:
1735:
1725:
1707:
1683:
1677:
1676:
1639:
1633:
1632:
1622:
1590:
1584:
1583:
1573:
1541:
1532:
1531:
1521:
1497:
1491:
1490:
1472:
1455:(9–10): 825–42.
1440:
1434:
1433:
1405:
1396:
1395:
1385:
1375:
1351:
1338:
1337:
1330:
1324:
1323:
1312:
1306:
1305:
1294:
1288:
1278:
1267:
1257:
1028:
1027:
999:
992:
975:
963:
954:
934:
930:RefSeq (protein)
920:
908:
899:
875:
851:
828:
802:
783:
757:
738:
707:
532:
518:
509:
502:
495:
488:
473:
449:seminal vesicula
445:genital tubercle
413:
411:Top expressed in
406:
395:caput epididymis
351:
349:Top expressed in
344:
323:
306:
296:
283:
272:
257:
250:
244:
233:
221:
205:
195:
182:
171:
156:
149:
143:
132:
118:
102:
96:
47:
40:
19:
2051:
2050:
2046:
2045:
2044:
2042:
2041:
2040:
2021:
2020:
2019:
2018:
1957:
1955:
1925:
1895:(10): 1546–60.
1889:Genome Research
1882:
1839:
1835:
1833:Further reading
1830:
1829:
1793:
1792:
1788:
1744:
1743:
1739:
1685:
1684:
1680:
1641:
1640:
1636:
1592:
1591:
1587:
1543:
1542:
1535:
1506:Cell Metabolism
1499:
1498:
1494:
1442:
1441:
1437:
1407:
1406:
1399:
1353:
1352:
1341:
1332:
1331:
1327:
1314:
1313:
1309:
1296:
1295:
1291:
1279:
1270:
1258:
1249:
1244:
1231:
1207:
1160:skeletal muscle
1152:
1113:
1089:
1084:
1037:View/Edit Mouse
1032:View/Edit Human
995:
988:
985:Location (UCSC)
971:
950:
946:
942:
916:
895:
891:
887:
883:
839:
792:ENSG00000136758
685:
629:
593:
584:protein binding
514:
504:
503:
497:
496:
469:
460:
455:
451:
447:
443:
439:
435:
431:
427:
423:
409:
398:
393:
389:
385:
381:
377:
373:
371:visceral pleura
369:
367:parietal pleura
365:
361:
347:
291:
278:
270:
260:
259:
258:
251:
231:
208:Gene location (
190:
177:
169:
159:
158:
157:
150:
128:
105:Gene location (
56:
43:
36:
17:
12:
11:
5:
2049:
2047:
2039:
2038:
2033:
2023:
2022:
2017:
2016:
2009:
2002:
1994:
1991:
1990:
1969:
1954:
1953:
1935:(2): 181–190.
1923:
1880:
1836:
1834:
1831:
1828:
1827:
1786:
1757:(6): 1010–23.
1737:
1678:
1634:
1585:
1533:
1492:
1435:
1397:
1339:
1325:
1307:
1289:
1268:
1246:
1245:
1243:
1240:
1239:
1238:
1235:
1230:
1227:
1206:
1203:
1151:
1148:
1112:
1109:
1097:chromosome 10p
1088:
1085:
1083:
1080:
1044:
1043:
1040:
1039:
1034:
1024:
1023:
1017:
1016:
1013:
1012:
1010:
1008:
1001:
1000:
993:
986:
982:
981:
978:
977:
967:
966:
960:
957:
956:
938:
937:
931:
927:
926:
923:
922:
912:
911:
905:
902:
901:
879:
878:
872:
868:
867:
864:
863:
855:
854:
848:
845:
844:
832:
831:
825:
819:
818:
815:
814:
806:
805:
799:
796:
795:
787:
786:
780:
774:
773:
770:
769:
761:
760:
754:
751:
750:
742:
741:
735:
729:
728:
723:
718:
714:
713:
703:
702:
699:
698:
687:
686:
684:
683:
678:
673:
668:
663:
658:
653:
648:
643:
637:
635:
631:
630:
628:
627:
622:
617:
612:
607:
601:
599:
595:
594:
592:
591:
586:
581:
576:
571:
566:
561:
556:
551:
545:
543:
539:
538:
528:
527:
524:
523:
520:
519:
511:
510:
485:
479:
478:
475:
474:
466:
465:
462:
461:
459:
458:
454:
450:
446:
442:
441:tail of embryo
438:
434:
430:
426:
422:
418:
415:
414:
403:
400:
399:
397:
396:
392:
388:
384:
380:
376:
375:amniotic fluid
372:
368:
364:
360:
356:
353:
352:
340:
339:
331:
320:
314:
313:
310:RNA expression
302:
301:
298:
297:
289:
285:
284:
276:
273:
268:
262:
261:
252:
245:
239:
235:
234:
229:
223:
222:
214:
213:
201:
200:
197:
196:
188:
184:
183:
175:
172:
167:
161:
160:
151:
144:
138:
134:
133:
126:
120:
119:
111:
110:
98:
97:
54:
50:
49:
41:
33:
32:
28:
27:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
2048:
2037:
2034:
2032:
2029:
2028:
2026:
2015:
2010:
2008:
2003:
2001:
1996:
1995:
1989:
1987:
1983:
1979:
1978:chromosome 10
1975:
1970:
1967:
1963:
1958:
1950:
1946:
1942:
1938:
1934:
1930:
1924:
1920:
1916:
1911:
1906:
1902:
1898:
1894:
1890:
1886:
1881:
1877:
1873:
1869:
1865:
1860:
1855:
1852:(3): 267–70.
1851:
1847:
1843:
1838:
1837:
1832:
1823:
1819:
1814:
1809:
1805:
1801:
1797:
1790:
1787:
1782:
1778:
1773:
1768:
1764:
1760:
1756:
1752:
1748:
1741:
1738:
1733:
1729:
1724:
1719:
1715:
1711:
1706:
1701:
1697:
1693:
1689:
1682:
1679:
1674:
1670:
1666:
1662:
1658:
1654:
1650:
1646:
1638:
1635:
1630:
1626:
1621:
1616:
1612:
1608:
1605:(6): 919–29.
1604:
1600:
1596:
1589:
1586:
1581:
1577:
1572:
1567:
1563:
1559:
1556:(1): 97–106.
1555:
1551:
1547:
1540:
1538:
1534:
1529:
1525:
1520:
1515:
1512:(2): 287–95.
1511:
1507:
1503:
1496:
1493:
1488:
1484:
1480:
1476:
1471:
1466:
1462:
1458:
1454:
1450:
1446:
1439:
1436:
1431:
1427:
1423:
1419:
1415:
1411:
1404:
1402:
1398:
1393:
1389:
1384:
1379:
1374:
1369:
1365:
1361:
1357:
1350:
1348:
1346:
1344:
1340:
1335:
1329:
1326:
1321:
1317:
1311:
1308:
1303:
1299:
1293:
1290:
1286:
1282:
1277:
1275:
1273:
1269:
1265:
1261:
1256:
1254:
1252:
1248:
1241:
1236:
1233:
1232:
1228:
1226:
1224:
1220:
1216:
1212:
1204:
1202:
1200:
1196:
1192:
1188:
1184:
1180:
1176:
1172:
1168:
1165:
1161:
1157:
1149:
1147:
1145:
1141:
1138:typical of a
1137:
1133:
1129:
1125:
1122:
1118:
1110:
1108:
1106:
1102:
1098:
1094:
1086:
1081:
1079:
1077:
1073:
1069:
1065:
1061:
1058:
1054:
1050:
1038:
1033:
1029:
1025:
1022:
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983:
976:
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928:
921:
919:
913:
909:
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890:
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880:
876:
873:
871:RefSeq (mRNA)
869:
862:
861:
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849:
843:
842:
838:
833:
829:
826:
824:
820:
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623:
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610:mitochondrion
608:
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541:
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536:Gene ontology
533:
529:
517:
512:
508:
501:
494:
489:
486:
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480:
472:
467:
456:
453:Gonadal ridge
452:
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436:
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394:
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46:
39:
34:
29:
25:
20:
1986:expanding it
1971:
1956:
1932:
1928:
1892:
1888:
1849:
1846:FEBS Letters
1845:
1803:
1799:
1789:
1754:
1750:
1740:
1698:(10): 2775.
1695:
1691:
1681:
1648:
1644:
1637:
1602:
1598:
1588:
1553:
1550:EMBO Reports
1549:
1509:
1505:
1495:
1452:
1448:
1438:
1416:(1): 48–54.
1413:
1409:
1363:
1359:
1328:
1319:
1310:
1301:
1292:
1229:Interactions
1221:affects the
1208:
1193:resistance,
1153:
1114:
1092:
1090:
1056:
1048:
1047:
970:
944:NP_001240795
941:
915:
885:NM_001253866
882:
858:
835:
809:
790:
764:
745:
725:
720:
620:nuclear body
421:pineal gland
333:
326:
53:External IDs
1136:HEXXH motif
1117:amino acids
646:proteolysis
569:ATP binding
292:23,089,272
279:23,046,381
271:2|2 A3
191:27,155,266
178:27,110,111
31:Identifiers
2025:Categories
1287:, May 2017
1266:, May 2017
1242:References
1211:homozygous
1064:AAA family
337:(ortholog)
74:HomoloGene
1976:on human
1714:2073-4409
1191:apoptotic
1082:Structure
1072:Mutations
973:NP_038799
952:NP_647473
948:NP_055078
918:NM_013771
897:NM_139313
893:NM_139312
889:NM_014263
711:Orthologs
433:spermatid
82:GeneCards
1949:12214059
1919:11042152
1876:42014862
1868:10930580
1822:27189080
1781:22262461
1732:34685755
1673:34322149
1665:26785494
1629:24616225
1580:25433032
1528:23931759
1479:11212342
1470:11146755
1430:10843804
1410:Genomics
1392:27495975
1283:–
1262:–
1164:pancreas
1150:Function
1134:, and a
1105:isoforms
1021:Wikidata
690:Sources:
615:membrane
457:gastrula
425:yolk sac
1772:3302729
1723:8534651
1645:Science
1620:3998800
1571:4304733
1487:5825456
1383:4991934
1285:Ensembl
1264:Ensembl
1215:atrophy
1111:Protein
1068:ATPases
823:UniProt
778:Ensembl
717:Species
696:QuickGO
312:pattern
170:10p12.1
70:1351651
38:Aliases
1947:
1917:
1910:310934
1907:
1874:
1866:
1820:
1779:
1769:
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1712:
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1627:
1617:
1578:
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1526:
1485:
1477:
1467:
1428:
1390:
1380:
1217:. The
1197:, and
1162:, and
1144:domain
1093:YME1L1
1057:YME1L1
1053:enzyme
1051:is an
1007:search
1005:PubMed
860:O88967
841:Q5T8D2
837:Q96TA2
733:Entrez
483:BioGPS
86:YME1L1
62:607472
45:YME1L1
22:YME1L1
1980:is a
1872:S2CID
1692:Cells
1669:S2CID
1483:S2CID
1360:eLife
1237:OMA1
1234:OPA1
1124:Yme1p
1121:yeast
1101:exons
766:27377
747:10730
726:Mouse
721:Human
692:Amigo
363:tibia
335:Mouse
328:Human
275:Start
210:Mouse
174:Start
107:Human
78:31996
1982:stub
1974:gene
1945:PMID
1915:PMID
1864:PMID
1818:PMID
1777:PMID
1728:PMID
1710:ISSN
1661:PMID
1625:PMID
1576:PMID
1524:PMID
1475:PMID
1426:PMID
1388:PMID
1179:OMA1
1175:OPA1
1140:zinc
1091:The
1087:Gene
1060:gene
318:Bgee
266:Band
227:Chr.
165:Band
124:Chr.
58:OMIM
1937:doi
1905:PMC
1897:doi
1854:doi
1850:478
1808:doi
1804:129
1767:PMC
1759:doi
1718:PMC
1700:doi
1653:doi
1649:350
1615:PMC
1607:doi
1603:204
1566:PMC
1558:doi
1514:doi
1465:PMC
1457:doi
1418:doi
1378:PMC
1368:doi
1223:MPP
1167:RNA
1066:of
288:End
187:End
90:OMA
66:MGI
2027::
1943:.
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1903:.
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1552:.
1548:.
1536:^
1522:.
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1508:.
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1481:.
1473:.
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1453:56
1451:.
1447:.
1424:.
1414:66
1412:.
1400:^
1386:.
1376:.
1366:.
1362:.
1358:.
1342:^
1318:.
1300:.
1271:^
1250:^
1209:A
1201:.
1146:.
1078:.
694:/
294:bp
281:bp
193:bp
180:bp
88:;
84::
80:;
76::
72:;
68::
64:;
60::
2013:e
2006:t
1999:v
1988:.
1951:.
1939::
1933:3
1921:.
1899::
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1856::
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1702::
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1432:.
1420::
1394:.
1370::
1364:5
1336:.
1322:.
1304:.
212:)
109:)
92::
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