353:, a part of an animal cell, helps break down large molecules into smaller ones that can be reused. GlcNAc-1-phosphotransferase phosphorylates carbon 6 of one or more mannosyl residues of N linked glycoproteins being processed in Golgi Apparatus . UDP-GLcNAc provides the phosphate in a reaction catalysed by this enzyme. M6P acts as an indicator of whether a hydrolase should be transported to the lysosome or not. Once a hydrolase indicates an M6P, it can be transported to a lysosome. Surprisingly some lysosomal enzymes are only tagged at a rate of 5% or lower.
1670:
483:
Kang, C., Riazuddin, S., Mundorff, J., Krasnewich, D., Friedman, P., Mullikin, J.C., and Drayna, D. (2010). Mutations in the
Lysosomal Enzyme–Targeting Pathway and Persistent Stuttering. New England Journal of Medicine 362, 677–685.
1318:
1328:
1510:
1323:
1443:
1421:
1239:
349:
produces the gamma subunit. GlcNAc-1-phosphotransferase functions to prepare newly made enzymes for lysosome transportation (lysosomal hydrolases to the lysosome).
387:
1503:
204:
59:
1244:
1496:
1411:
1735:
1730:
1711:
1050:
421:
406:
228:
83:
1184:
754:
548:
895:
216:
71:
209:
64:
1468:
1463:
1023:
841:
1333:
1221:
1189:
1174:
508:
856:
373:
1704:
1554:
1454:
1432:
1407:
1382:
1302:
910:
870:
815:
792:
764:
732:
598:
685:
919:
900:
831:
565:
518:
512:
1488:
1604:
1523:
777:
1231:
1004:
914:
221:
76:
503:
493:
1740:
1085:
1040:
742:
727:
663:
643:
541:
498:
274:
129:
526:
1697:
999:
994:
874:
737:
717:
626:
1640:
1262:
1254:
1035:
702:
638:
602:
1575:
1125:
1063:
1014:
749:
576:
468:
1592:
1140:
1130:
1120:
1045:
1029:
988:
974:
969:
707:
670:
460:
1681:
1669:
1597:
1473:
1135:
1115:
1057:
953:
851:
836:
782:
680:
675:
534:
450:
278:
269:
133:
124:
1544:
1396:
1386:
1208:
1179:
805:
712:
697:
298:
153:
17:
1580:
1364:
1203:
1110:
1102:
979:
942:
366:
1724:
1570:
633:
455:
438:
472:
1527:
722:
692:
621:
245:
100:
1614:
1586:
1288:
1278:
879:
616:
557:
328:
308:
163:
1609:
1519:
1090:
933:
846:
611:
561:
252:
107:
1369:
1282:
819:
585:
339:
It is made up of two alpha (α), two betas (β), and two gammas (γ) subunits.
464:
1216:
796:
350:
257:
112:
521:
439:"Novel MITF targets identified using a two-step DNA microarray strategy"
1630:
1167:
1152:
768:
1677:
1530:
1145:
580:
240:
95:
33:
N-acetylglucosamine-1-phosphate transferase, alpha and beta subunits
1162:
1157:
1009:
345:
1319:
CDP-diacylglycerol—glycerol-3-phosphate 3-phosphatidyltransferase
1549:
1073:
1068:
964:
959:
658:
653:
648:
425:
410:
386:
In melanocytic cells, GNPTG gene expression may be regulated by
233:
88:
1492:
530:
494:
178:
N-acetylglucosamine-1-phosphate transferase, gamma subunit
504:
1685:
437:
Hoek KS, Schlegel NC, Eichhoff OM, et al. (2008).
1329:
CDP-diacylglycerol—inositol 3-phosphatidyltransferase
1334:
CDP-diacylglycerol—choline O-phosphatidyltransferase
1639:
1623:
1563:
1537:
1453:
1431:
1406:
1381:
1362:
1342:
1324:
CDP-diacylglycerol—serine O-phosphatidyltransferase
1311:
1301:
1271:
1253:
1230:
1202:
1101:
941:
932:
909:
869:
814:
791:
763:
597:
573:
304:
294:
289:
268:
263:
251:
239:
227:
215:
203:
195:
187:
182:
177:
159:
149:
144:
123:
118:
106:
94:
82:
70:
58:
50:
42:
37:
32:
499:GeneReviews/NIH/NCBI/UW entry on Mucolipidosis II
361:It is associated with the following conditions:
1705:
1504:
542:
8:
1650:N-acetylglucosamine-1-phosphate transferase
1350:N-acetylglucosamine-1-phosphate transferase
1240:UTP—glucose-1-phosphate uridylyltransferase
509:N-acetylglucosamine-1-phosphate+transferase
321:N-acetylglucosamine-1-phosphate transferase
1712:
1698:
1511:
1497:
1489:
1378:
1308:
938:
929:
594:
549:
535:
527:
286:
141:
1444:serine/threonine-specific protein kinases
1422:serine/threonine-specific protein kinases
1245:Galactose-1-phosphate uridylyltransferase
511:at the U.S. National Library of Medicine
454:
399:
27:Mammalian protein found in Homo sapiens
343:produces the alpha and beta subunits,
174:
29:
7:
1666:
1664:
411:MUCOLIPIDOSIS II ALPHA/BETA - 252500
422:Online Mendelian Inheritance in Man
407:Online Mendelian Inheritance in Man
1684:. You can help Knowledge (XXG) by
25:
755:Glucose-1,6-bisphosphate synthase
1668:
896:Ribose-phosphate diphosphokinase
456:10.1111/j.1755-148X.2008.00505.x
426:MUCOLIPIDOSIS III GAMMA - 252605
379:mucolipidosis III gamma - GNPTG
382:stuttering (Kang et al., 2010)
372:mucolipidosis III alpha/beta (
1:
1469:Protein-histidine tele-kinase
1464:Protein-histidine pros-kinase
1343:Glycosyl-1-phosphotransferase
365:mucolipidosis II alpha/beta (
1736:Genes on human chromosome 16
1731:Genes on human chromosome 12
1175:RNA-dependent RNA polymerase
1082:RNA-directed DNA polymerase
950:DNA-directed DNA polymerase
374:pseudo-Hurler polydystrophy
1757:
1663:
1435:: protein-dual-specificity
325:GlcNAc-1-phosphotrasferase
1555:Oligosaccharyltransferase
443:Pigment Cell Melanoma Res
285:
140:
18:GlcNAc phosphotransferase
1412:protein-serine/threonine
1312:Phosphatidyltransferases
901:Thiamine diphosphokinase
513:Medical Subject Headings
1605:Aspartylglucosaminidase
1524:carbohydrate metabolism
1680:-related article is a
1232:Nucleotidyltransferase
915:nucleotidyltransferase
842:Nucleoside-diphosphate
1086:Reverse transcriptase
357:Clinical significance
875:diphosphotransferase
857:Thiamine-diphosphate
564:-containing groups (
1457:: protein-histidine
1375:; protein acceptor)
1263:mRNA capping enzyme
1255:Guanylyltransferase
1576:Beta-galactosidase
733:Phosphoinositide 3
577:phosphotransferase
1693:
1692:
1658:
1657:
1593:alpha-Mannosidase
1486:
1485:
1482:
1481:
1358:
1357:
1297:
1296:
1198:
1197:
1111:Template-directed
865:
864:
832:Phosphomevalonate
318:
317:
314:
313:
173:
172:
169:
168:
16:(Redirected from
1748:
1714:
1707:
1700:
1672:
1665:
1598:beta-mannosidase
1513:
1506:
1499:
1490:
1474:Histidine kinase
1397:tyrosine kinases
1387:protein-tyrosine
1379:
1309:
1116:RNA polymerase I
939:
930:
783:Aspartate kinase
778:Phosphoglycerate
595:
551:
544:
537:
528:
477:
476:
458:
434:
428:
419:
413:
404:
287:
175:
142:
30:
21:
1756:
1755:
1751:
1750:
1749:
1747:
1746:
1745:
1721:
1720:
1719:
1718:
1661:
1659:
1654:
1635:
1619:
1559:
1545:Dolichol kinase
1533:
1517:
1487:
1478:
1449:
1427:
1402:
1373:
1367:
1363:2.7.10-2.7.13:
1354:
1338:
1305:: miscellaneous
1293:
1267:
1249:
1226:
1209:exoribonuclease
1206:
1194:
1180:Polyadenylation
1097:
923:
917:
905:
887:
883:
877:
861:
823:
810:
787:
759:
589:
583:
575:
569:
555:
490:
481:
480:
436:
435:
431:
420:
416:
405:
401:
396:
359:
337:
28:
23:
22:
15:
12:
11:
5:
1754:
1752:
1744:
1743:
1738:
1733:
1723:
1722:
1717:
1716:
1709:
1702:
1694:
1691:
1690:
1673:
1656:
1655:
1653:
1652:
1646:
1644:
1637:
1636:
1634:
1633:
1627:
1625:
1621:
1620:
1618:
1617:
1612:
1607:
1602:
1601:
1600:
1595:
1583:
1581:Hexosaminidase
1578:
1573:
1567:
1565:
1561:
1560:
1558:
1557:
1552:
1547:
1541:
1539:
1535:
1534:
1518:
1516:
1515:
1508:
1501:
1493:
1484:
1483:
1480:
1479:
1477:
1476:
1471:
1466:
1460:
1458:
1451:
1450:
1448:
1447:
1438:
1436:
1429:
1428:
1426:
1425:
1416:
1414:
1404:
1403:
1401:
1400:
1391:
1389:
1376:
1371:
1365:protein kinase
1360:
1359:
1356:
1355:
1353:
1352:
1346:
1344:
1340:
1339:
1337:
1336:
1331:
1326:
1321:
1315:
1313:
1306:
1299:
1298:
1295:
1294:
1292:
1291:
1286:
1275:
1273:
1269:
1268:
1266:
1265:
1259:
1257:
1251:
1250:
1248:
1247:
1242:
1236:
1234:
1228:
1227:
1225:
1224:
1219:
1213:
1211:
1204:Phosphorolytic
1200:
1199:
1196:
1195:
1193:
1192:
1187:
1182:
1177:
1172:
1171:
1170:
1165:
1160:
1150:
1149:
1148:
1138:
1133:
1128:
1123:
1118:
1113:
1107:
1105:
1103:RNA polymerase
1099:
1098:
1096:
1095:
1094:
1093:
1083:
1079:
1078:
1077:
1076:
1071:
1066:
1055:
1054:
1053:
1048:
1043:
1038:
1027:
1021:
1020:
1019:
1012:
1007:
1002:
997:
986:
985:
984:
977:
972:
967:
962:
951:
947:
945:
943:DNA polymerase
936:
927:
921:
907:
906:
904:
903:
898:
892:
890:
885:
881:
867:
866:
863:
862:
860:
859:
854:
849:
844:
839:
834:
828:
826:
821:
812:
811:
809:
808:
802:
800:
789:
788:
786:
785:
780:
774:
772:
761:
760:
758:
757:
752:
747:
746:
745:
740:
730:
728:Diacylglycerol
725:
720:
715:
710:
705:
700:
695:
690:
689:
688:
678:
673:
668:
667:
666:
661:
656:
651:
646:
639:Phosphofructo-
636:
631:
630:
629:
619:
614:
608:
606:
592:
587:
571:
570:
556:
554:
553:
546:
539:
531:
525:
524:
516:
506:
501:
496:
489:
488:External links
486:
479:
478:
429:
414:
398:
397:
395:
392:
384:
383:
380:
377:
370:
367:I-cell disease
358:
355:
336:
333:
316:
315:
312:
311:
306:
302:
301:
296:
292:
291:
283:
282:
272:
266:
265:
261:
260:
255:
249:
248:
243:
237:
236:
231:
225:
224:
219:
213:
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207:
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197:
193:
192:
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185:
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179:
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167:
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161:
157:
156:
151:
147:
146:
138:
137:
127:
121:
120:
116:
115:
110:
104:
103:
98:
92:
91:
86:
80:
79:
74:
68:
67:
62:
56:
55:
52:
48:
47:
44:
40:
39:
35:
34:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
1753:
1742:
1739:
1737:
1734:
1732:
1729:
1728:
1726:
1715:
1710:
1708:
1703:
1701:
1696:
1695:
1689:
1687:
1683:
1679:
1674:
1671:
1667:
1662:
1651:
1648:
1647:
1645:
1642:
1638:
1632:
1629:
1628:
1626:
1622:
1616:
1613:
1611:
1608:
1606:
1603:
1599:
1596:
1594:
1591:
1590:
1589:
1588:
1584:
1582:
1579:
1577:
1574:
1572:
1571:Neuraminidase
1569:
1568:
1566:
1562:
1556:
1553:
1551:
1548:
1546:
1543:
1542:
1540:
1536:
1532:
1529:
1525:
1521:
1514:
1509:
1507:
1502:
1500:
1495:
1494:
1491:
1475:
1472:
1470:
1467:
1465:
1462:
1461:
1459:
1456:
1452:
1446:
1445:
1440:
1439:
1437:
1434:
1430:
1424:
1423:
1418:
1417:
1415:
1413:
1409:
1405:
1399:
1398:
1393:
1392:
1390:
1388:
1384:
1380:
1377:
1374:
1366:
1361:
1351:
1348:
1347:
1345:
1341:
1335:
1332:
1330:
1327:
1325:
1322:
1320:
1317:
1316:
1314:
1310:
1307:
1304:
1300:
1290:
1287:
1284:
1280:
1277:
1276:
1274:
1270:
1264:
1261:
1260:
1258:
1256:
1252:
1246:
1243:
1241:
1238:
1237:
1235:
1233:
1229:
1223:
1220:
1218:
1215:
1214:
1212:
1210:
1205:
1201:
1191:
1188:
1186:
1183:
1181:
1178:
1176:
1173:
1169:
1166:
1164:
1161:
1159:
1156:
1155:
1154:
1151:
1147:
1144:
1143:
1142:
1139:
1137:
1134:
1132:
1129:
1127:
1124:
1122:
1119:
1117:
1114:
1112:
1109:
1108:
1106:
1104:
1100:
1092:
1089:
1088:
1087:
1084:
1081:
1080:
1075:
1072:
1070:
1067:
1065:
1062:
1061:
1059:
1056:
1052:
1049:
1047:
1044:
1042:
1039:
1037:
1034:
1033:
1031:
1028:
1025:
1022:
1018:
1017:
1013:
1011:
1008:
1006:
1003:
1001:
998:
996:
993:
992:
990:
987:
983:
982:
978:
976:
973:
971:
968:
966:
963:
961:
958:
957:
955:
952:
949:
948:
946:
944:
940:
937:
935:
931:
928:
925:
916:
912:
908:
902:
899:
897:
894:
893:
891:
888:
876:
872:
868:
858:
855:
853:
850:
848:
845:
843:
840:
838:
835:
833:
830:
829:
827:
824:
817:
813:
807:
804:
803:
801:
798:
794:
790:
784:
781:
779:
776:
775:
773:
770:
766:
762:
756:
753:
751:
748:
744:
743:Class II PI 3
741:
739:
736:
735:
734:
731:
729:
726:
724:
721:
719:
718:Deoxycytidine
716:
714:
711:
709:
706:
704:
701:
699:
696:
694:
691:
687:
686:ADP-thymidine
684:
683:
682:
679:
677:
674:
672:
669:
665:
662:
660:
657:
655:
652:
650:
647:
645:
642:
641:
640:
637:
635:
632:
628:
625:
624:
623:
620:
618:
615:
613:
610:
609:
607:
604:
600:
596:
593:
590:
582:
578:
572:
567:
563:
559:
552:
547:
545:
540:
538:
533:
532:
529:
523:
520:
517:
514:
510:
507:
505:
502:
500:
497:
495:
492:
491:
487:
485:
474:
470:
466:
462:
457:
452:
449:(6): 665–76.
448:
444:
440:
433:
430:
427:
423:
418:
415:
412:
408:
403:
400:
393:
391:
389:
381:
378:
375:
371:
368:
364:
363:
362:
356:
354:
352:
348:
347:
342:
334:
332:
330:
326:
322:
310:
307:
303:
300:
297:
293:
288:
284:
281:
280:
276:
273:
271:
267:
262:
259:
256:
254:
250:
247:
244:
242:
238:
235:
232:
230:
226:
223:
220:
218:
214:
211:
208:
206:
202:
198:
194:
190:
186:
181:
176:
165:
162:
158:
155:
152:
148:
143:
139:
136:
135:
131:
128:
126:
122:
117:
114:
111:
109:
105:
102:
99:
97:
93:
90:
87:
85:
81:
78:
75:
73:
69:
66:
63:
61:
57:
53:
49:
45:
41:
36:
31:
19:
1741:Enzyme stubs
1686:expanding it
1675:
1660:
1649:
1585:
1528:glycoprotein
1441:
1419:
1394:
1349:
1015:
980:
738:Class I PI 3
703:Pantothenate
574:2.7.1-2.7.4:
558:Transferases
482:
446:
442:
432:
417:
402:
385:
360:
344:
340:
338:
324:
320:
319:
277:
196:Alt. symbols
132:
51:Alt. symbols
1587:mannosidase
1289:Transposase
1279:Recombinase
924:-nucleoside
750:Sphingosine
329:transferase
299:Swiss-model
183:Identifiers
154:Swiss-model
38:Identifiers
1725:Categories
1610:Fucosidase
1564:Catabolism
1520:Metabolism
1091:Telomerase
934:Polymerase
708:Mevalonate
671:Riboflavin
562:phosphorus
394:References
376:) - GNPTAB
369:) - GNPTAB
295:Structures
290:Search for
264:Other data
150:Structures
145:Search for
119:Other data
1624:Transport
1538:Anabolism
1283:Integrase
1207:3' to 5'
852:Guanylate
847:Uridylate
837:Adenylate
681:Thymidine
676:Shikimate
351:Lysosomes
246:NM_032520
205:NCBI gene
101:NM_024312
60:NCBI gene
1217:RNase PH
825:acceptor
806:Creatine
799:acceptor
771:acceptor
713:Pyruvate
698:Glycerol
659:Platelet
634:Galacto-
605:acceptor
522:2.7.8.15
473:24698373
465:19067971
424:(OMIM):
409:(OMIM):
335:Function
331:enzyme.
309:InterPro
164:InterPro
1643:tagging
1631:SLC17A5
1531:enzymes
1526:·
1168:PrimPol
1153:Primase
627:Hepatic
622:Fructo-
327:) is a
305:Domains
275:Chr. 16
253:UniProt
160:Domains
130:Chr. 12
108:UniProt
1678:enzyme
1455:2.7.13
1433:2.7.12
1408:2.7.11
1383:2.7.10
1222:PNPase
1190:PNPase
1146:POLRMT
1141:ssRNAP
654:Muscle
617:Gluco-
581:kinase
515:(MeSH)
471:
463:
341:GNPTAB
258:Q9UJJ9
241:RefSeq
234:607838
199:GNPTAG
188:Symbol
113:Q3T906
96:RefSeq
89:607840
46:GNPTAB
43:Symbol
1676:This
1303:2.7.8
1272:Other
911:2.7.7
871:2.7.6
816:2.7.4
793:2.7.3
765:2.7.2
649:Liver
612:Hexo-
599:2.7.1
469:S2CID
346:GNPTG
279:p13.3
270:Locus
222:23026
210:84572
191:GNPTG
134:q23.3
125:Locus
77:29670
65:79158
54:GNPTA
1682:stub
1615:NAGA
1550:GCS1
1442:see
1420:see
1395:see
769:COOH
568:2.7)
461:PMID
388:MITF
229:OMIM
217:HGNC
84:OMIM
72:HGNC
1641:M6P
1185:PAP
1126:III
1060:/Y
1051:TDT
1032:/X
1024:III
1016:Pfu
991:/B
981:Taq
956:/A
723:PFP
693:NAD
451:doi
1727::
1522::
1410::
1385::
1370:PO
1131:IV
1121:II
1030:IV
1026:/C
989:II
975:T7
920:PO
913::
873::
820:PO
818::
795::
767::
603:OH
601::
586:PO
566:EC
560::
519:EC
467:.
459:.
447:21
445:.
441:.
390:.
1713:e
1706:t
1699:v
1688:.
1512:e
1505:t
1498:v
1372:4
1368:(
1285:)
1281:(
1163:2
1158:1
1136:V
1074:Îş
1069:Îą
1064:η
1058:V
1046:ÎĽ
1041:λ
1036:β
1010:ζ
1005:ε
1000:δ
995:α
970:ν
965:θ
960:Îł
954:I
926:)
922:4
918:(
889:)
886:7
884:O
882:2
880:P
878:(
822:4
797:N
664:2
644:1
591:)
588:4
584:(
579:/
550:e
543:t
536:v
475:.
453::
323:(
20:)
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