159:
and is attached to the ER membrane by its hydrophobic C terminus; the majority of the protein extends into the ER lumen. The hydrophobic C-terminal sequence is then cleaved off and replaced by the GPI-anchor. As the protein processes through the
71:
390:
Krawitz PM, Höchsmann B, Murakami Y, Teubner B, Krüger U, Klopocki E, Neitzel H, Hoellein A, Schneider C, Parkhomchuk D, Hecht J, Robinson PN, Mundlos S, Kinoshita T, Schrezenmeier H (August 2013).
197:(PLC) is an enzyme known to cleave the phospho-glycerol bond found in GPI-anchored proteins. Treatment with PLC will cause release of GPI-linked proteins from the outer cell membrane. The
504:
475:
235:
1486:
1642:
1342:
497:
1476:
1468:
1460:
231:
490:
213:, are known to be GPI-linked and are released by treatment with PLC. GPI-linked proteins are thought to be preferentially located in
432:
1447:
1442:
1437:
517:
210:
100:
433:"Localization of a Variable Surface Glycoprotein Phosphatidylinositol-Specific Phospholipase-C in Trypanosoma brucei brucei"
238:(HPMRS). In PNH a somatic defect in blood stem cells, which is required for GPI synthesis, results in faulty GPI linkage of
311:
1167:
1335:
1513:
1637:
1415:
1207:
618:
513:
266:
and a second acquired somatic hit has also been reported. Without these proteins linked to the cell surface, the
239:
482:
1390:
468:
1261:
392:"A case of paroxysmal nocturnal hemoglobinuria caused by a germline mutation and a somatic mutation in PIGT"
1145:
1328:
128:
464:
1589:
152:
1410:
1405:
536:
202:
108:
104:
551:
1395:
1369:
623:
136:
51:
972:
1571:
1400:
962:
866:
716:
320:
180:
will result in controlled release of the protein from the membrane. The latter mechanism is used
1381:
820:
706:
665:
413:
372:
343:"The glycosylphosphatidylinositol anchor: a complex membrane-anchoring structure for proteins"
315:
267:
257:
161:
186:; i.e. membrane proteins released from membranes in enzymatic assays are glypiated proteins.
1123:
1101:
1000:
919:
810:
787:
711:
683:
403:
362:
354:
88:
298:
292:
176:
is the sole means of attachment of such proteins to the membrane, cleavage of the group by
1234:
934:
825:
701:
660:
286:
194:
165:
1607:
1096:
990:
967:
909:
759:
741:
628:
594:
440:
367:
342:
275:
247:
148:
1631:
1551:
1115:
1060:
1042:
1018:
957:
952:
904:
899:
848:
805:
726:
670:
574:
278:. For patients with HPMRS, disease-causing mutations have been reported in the genes
177:
169:
1355:
914:
731:
546:
531:
112:
56:
408:
391:
168:
and finally to the plasma membrane where it remains attached to a leaflet of the
1542:
1528:
1508:
1499:
1036:
995:
924:
889:
884:
843:
815:
782:
764:
736:
589:
579:
569:
541:
132:
116:
250:. The most common cause of PNH are somatic mutations in the X-chromosomal gene
217:, suggesting a high level of organization within plasma membrane microdomains.
107:
play key roles in a wide variety of biological processes. GPI is composed of a
1612:
1276:
1182:
1159:
1155:
1111:
1075:
1052:
835:
639:
605:
561:
214:
173:
156:
92:
135:
within the hydrophobic phosphatidyl-inositol group anchor the protein to the
1305:
1218:
1028:
947:
894:
858:
797:
774:
721:
652:
584:
230:
Defects in the GPI-anchor synthesis occur in rare acquired diseases such as
417:
376:
439:. Food and Agricultural Organization of the United Nations. Archived from
155:(ER). The protein is co-translationally inserted in the ER membrane via a
17:
1576:
1363:
1293:
1289:
1285:
1222:
1195:
1137:
1088:
1084:
982:
929:
751:
693:
675:
182:
124:
1320:
131:(EtNP) bridge to the C-terminal amino acid of a mature protein. The two
1602:
1253:
1245:
1226:
1199:
1010:
120:
96:
358:
1297:
1249:
1191:
1133:
876:
648:
198:
1351:
942:
613:
271:
280:
262:
252:
243:
206:
1324:
486:
274:
the cell, and high numbers of RBCs are destroyed, leading to
1235:
4-(p-hydroxybenzylidene)-5-imidazolinone (HBI) formation
324:
are attached to the plasma membrane via a GPI anchor.
1560:
1541:
1498:
1427:
1380:
1362:
1284:
1274:
1244:
1217:
1190:
1180:
1154:
1132:
1110:
1083:
1073:
1051:
1027:
1009:
981:
875:
857:
834:
796:
773:
750:
692:
647:
637:
604:
560:
524:
50:
42:
37:
32:
1208:p-Hydroxybenzylidene-imidazolinone (HBI) formation
236:hyperphosphatasia with mental retardation syndrome
147:Glycosylated (GPI-anchored) proteins contain a
1336:
498:
8:
1168:Tryptophan tryptophylquinone (TTQ) formation
1564:
1377:
1343:
1329:
1321:
1281:
1187:
1080:
644:
505:
491:
483:
467:at the U.S. National Library of Medicine
407:
366:
164:, it is transferred via vesicles to the
1262:Methylidene-imidazolone (MIO) formation
333:
234:(PNH) and congenital diseases such as
29:
1146:Lysine tyrosylquinone (LTQ) formation
341:Paulick MG, Bertozzi CR (July 2008).
27:Phosphoglyceride attached to proteins
7:
619:Glycosyl phosphatidylinositol (GPI)
232:paroxysmal nocturnal hemoglobinuria
25:
930:Oxidative deamination to aldehyde
437:FAO Corporate document depository
221:GPI-anchor synthesis deficiencies
1643:Post-translational modification
518:posttranslational modifications
211:placental alkaline phosphatases
312:variable surface glycoproteins
101:posttranslational modification
1:
465:Glycosylphosphatidylinositols
256:. However, a PNH case with a
151:, thus directing them to the
409:10.1182/blood-2013-01-481499
123:glycosidically bound to the
91:that can be attached to the
65:Glycosylphosphatidylinositol
1391:-ethanolamine/cephalin (PE)
737:Topaquinone (TPQ) formation
1659:
1514:Platelet-activating factor
1598:
1585:
1567:
1524:
1181:Crosslinks between three
514:Protein primary structure
240:decay-accelerating factor
81:glycophosphatidylinositol
1275:Crosslinks between four
469:Medical Subject Headings
33:GPI synthesis components
1074:Crosslinks between two
111:group linked through a
1396:-choline/lecithin (PC)
722:Porphyrin ring linkage
476:"Gpi Anchor Structure"
260:in the autosomal gene
129:ethanolamine phosphate
1590:Lysophosphatidic acid
1370:Glycerophospholipids/
783:Succinimide formation
153:endoplasmic reticulum
105:GPI-anchored proteins
537:Protein biosynthesis
203:acetylcholinesterase
127:residue) and via an
115:-containing linker (
109:phosphatidylinositol
431:Grab DJ, Verjee Y.
1572:Inositol phosphate
1428:Phosphoinositides:
867:Transglutamination
321:Trypanosoma brucei
205:, as well as both
1625:
1624:
1621:
1620:
1537:
1536:
1372:Phosphoglycerides
1318:
1317:
1314:
1313:
1270:
1269:
1176:
1175:
1069:
1068:
821:Polyglutamylation
707:Dephosphorylation
666:Dephosphorylation
359:10.1021/bi8006324
353:(27): 6991–7000.
316:sleeping sickness
268:complement system
258:germline mutation
201:marker Thy-1 and
162:secretory pathway
62:
61:
16:(Redirected from
1650:
1638:Membrane biology
1565:
1378:
1345:
1338:
1331:
1322:
1282:
1188:
1124:Sulfilimine bond
1102:ADP-ribosylation
1081:
1001:ADP-ribosylation
920:ADP-ribosylation
811:ADP-ribosylation
788:ADP-ribosylation
712:ADP-ribosylation
684:ADP-ribosylation
645:
638:Single specific
507:
500:
493:
484:
479:
478:. Sigma-Aldrich.
452:
451:
449:
448:
428:
422:
421:
411:
387:
381:
380:
370:
338:
306:In other species
103:. The resulting
89:phosphoglyceride
78:
77:
76:
74:
30:
21:
1658:
1657:
1653:
1652:
1651:
1649:
1648:
1647:
1628:
1627:
1626:
1617:
1594:
1581:
1556:
1533:
1520:
1494:
1490:
1480:
1472:
1464:
1457:
1423:
1371:
1367:
1358:
1349:
1319:
1310:
1266:
1240:
1213:
1172:
1150:
1128:
1106:
1065:
1061:C-mannosylation
1047:
1023:
1005:
977:
943:Imine formation
871:
853:
830:
826:Polyglycylation
792:
769:
746:
702:Phosphorylation
688:
661:Phosphorylation
633:
600:
556:
520:
511:
474:
461:
456:
455:
446:
444:
430:
429:
425:
389:
388:
384:
340:
339:
335:
330:
308:
248:red blood cells
228:
223:
195:Phospholipase C
192:
166:Golgi apparatus
149:signal sequence
145:
72:
70:
69:
68:
28:
23:
22:
15:
12:
11:
5:
1656:
1654:
1646:
1645:
1640:
1630:
1629:
1623:
1622:
1619:
1618:
1616:
1615:
1610:
1608:Phosphocholine
1605:
1599:
1596:
1595:
1593:
1592:
1586:
1583:
1582:
1580:
1579:
1574:
1568:
1562:
1558:
1557:
1555:
1554:
1548:
1546:
1539:
1538:
1535:
1534:
1532:
1531:
1525:
1522:
1521:
1519:
1518:
1517:
1516:
1505:
1503:
1496:
1495:
1493:
1492:
1488:
1484:
1483:
1482:
1478:
1474:
1470:
1466:
1462:
1455:
1452:
1451:
1450:
1445:
1440:
1431:
1429:
1425:
1424:
1422:
1421:
1420:
1419:
1411:-inositol (PI)
1408:
1406:-glycerol (PG)
1403:
1398:
1393:
1387:
1385:
1375:
1360:
1359:
1350:
1348:
1347:
1340:
1333:
1325:
1316:
1315:
1312:
1311:
1309:
1308:
1302:
1300:
1279:
1272:
1271:
1268:
1267:
1265:
1264:
1258:
1256:
1242:
1241:
1239:
1238:
1231:
1229:
1215:
1214:
1212:
1211:
1204:
1202:
1185:
1178:
1177:
1174:
1173:
1171:
1170:
1164:
1162:
1152:
1151:
1149:
1148:
1142:
1140:
1130:
1129:
1127:
1126:
1120:
1118:
1108:
1107:
1105:
1104:
1099:
1097:Disulfide bond
1093:
1091:
1078:
1071:
1070:
1067:
1066:
1064:
1063:
1057:
1055:
1049:
1048:
1046:
1045:
1040:
1033:
1031:
1025:
1024:
1022:
1021:
1015:
1013:
1007:
1006:
1004:
1003:
998:
993:
991:Citrullination
987:
985:
979:
978:
976:
975:
970:
968:Propionylation
965:
960:
955:
950:
945:
940:
938:-glycosylation
932:
927:
922:
917:
912:
910:Ubiquitination
907:
902:
897:
892:
887:
881:
879:
873:
872:
870:
869:
863:
861:
855:
854:
852:
851:
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840:
838:
832:
831:
829:
828:
823:
818:
813:
808:
802:
800:
794:
793:
791:
790:
785:
779:
777:
771:
770:
768:
767:
762:
760:Palmitoylation
756:
754:
748:
747:
745:
744:
742:Detyrosination
739:
734:
732:Flavin linkage
729:
724:
719:
714:
709:
704:
698:
696:
690:
689:
687:
686:
681:
673:
668:
663:
657:
655:
642:
635:
634:
632:
631:
629:Detyrosination
626:
621:
616:
610:
608:
602:
601:
599:
598:
595:Myristoylation
592:
587:
582:
577:
572:
566:
564:
558:
557:
555:
554:
552:N–O acyl shift
549:
544:
539:
534:
528:
526:
522:
521:
512:
510:
509:
502:
495:
487:
481:
480:
472:
460:
459:External links
457:
454:
453:
423:
382:
332:
331:
329:
326:
307:
304:
276:hemoglobinuria
227:
224:
222:
219:
191:
188:
178:phospholipases
144:
141:
60:
59:
54:
48:
47:
44:
40:
39:
35:
34:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
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1614:
1611:
1609:
1606:
1604:
1601:
1600:
1597:
1591:
1588:
1587:
1584:
1578:
1575:
1573:
1570:
1569:
1566:
1563:
1559:
1553:
1552:Sphingomyelin
1550:
1549:
1547:
1544:
1540:
1530:
1527:
1526:
1523:
1515:
1512:
1511:
1510:
1507:
1506:
1504:
1501:
1497:
1491:
1485:
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1475:
1473:
1467:
1465:
1459:
1458:
1453:
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1446:
1444:
1441:
1439:
1436:
1435:
1433:
1432:
1430:
1426:
1417:
1414:
1413:
1412:
1409:
1407:
1404:
1402:
1399:
1397:
1394:
1392:
1389:
1388:
1386:
1383:
1382:Phosphatidyl-
1379:
1376:
1373:
1365:
1361:
1357:
1356:phospholipids
1353:
1346:
1341:
1339:
1334:
1332:
1327:
1326:
1323:
1307:
1304:
1303:
1301:
1299:
1295:
1291:
1287:
1283:
1280:
1278:
1273:
1263:
1260:
1259:
1257:
1255:
1251:
1247:
1243:
1237:(chromophore)
1236:
1233:
1232:
1230:
1228:
1224:
1220:
1216:
1210:(chromophore)
1209:
1206:
1205:
1203:
1201:
1197:
1193:
1189:
1186:
1184:
1179:
1169:
1166:
1165:
1163:
1161:
1157:
1153:
1147:
1144:
1143:
1141:
1139:
1135:
1131:
1125:
1122:
1121:
1119:
1117:
1116:Hydroxylysine
1113:
1109:
1103:
1100:
1098:
1095:
1094:
1092:
1090:
1086:
1082:
1079:
1077:
1072:
1062:
1059:
1058:
1056:
1054:
1050:
1044:
1043:Adenylylation
1041:
1038:
1035:
1034:
1032:
1030:
1026:
1020:
1019:Hydroxylation
1017:
1016:
1014:
1012:
1008:
1002:
999:
997:
994:
992:
989:
988:
986:
984:
980:
974:
971:
969:
966:
964:
961:
959:
958:Succinylation
956:
954:
953:Carbamylation
951:
949:
946:
944:
941:
939:
937:
933:
931:
928:
926:
923:
921:
918:
916:
913:
911:
908:
906:
905:Hydroxylation
903:
901:
900:Adenylylation
898:
896:
893:
891:
888:
886:
883:
882:
880:
878:
874:
868:
865:
864:
862:
860:
856:
850:
849:Glycosylation
847:
845:
842:
841:
839:
837:
833:
827:
824:
822:
819:
817:
814:
812:
809:
807:
806:Carboxylation
804:
803:
801:
799:
795:
789:
786:
784:
781:
780:
778:
776:
772:
766:
763:
761:
758:
757:
755:
753:
749:
743:
740:
738:
735:
733:
730:
728:
727:Adenylylation
725:
723:
720:
718:
715:
713:
710:
708:
705:
703:
700:
699:
697:
695:
691:
685:
682:
680:
678:
674:
672:
671:Glycosylation
669:
667:
664:
662:
659:
658:
656:
654:
650:
646:
643:
641:
636:
630:
627:
625:
624:O-methylation
622:
620:
617:
615:
612:
611:
609:
607:
603:
596:
593:
591:
588:
586:
583:
581:
578:
576:
575:Carbamylation
573:
571:
568:
567:
565:
563:
559:
553:
550:
548:
545:
543:
540:
538:
535:
533:
530:
529:
527:
523:
519:
515:
508:
503:
501:
496:
494:
489:
488:
485:
477:
473:
470:
466:
463:
462:
458:
443:on 2018-08-31
442:
438:
434:
427:
424:
419:
415:
410:
405:
402:(7): 1312–5.
401:
397:
393:
386:
383:
378:
374:
369:
364:
360:
356:
352:
348:
344:
337:
334:
327:
325:
323:
322:
317:
313:
305:
303:
301:
300:
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294:
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283:
282:
277:
273:
269:
265:
264:
259:
255:
254:
249:
245:
241:
237:
233:
225:
220:
218:
216:
212:
208:
204:
200:
196:
189:
187:
185:
184:
179:
175:
171:
170:cell membrane
167:
163:
158:
154:
150:
142:
140:
138:
137:cell membrane
134:
130:
126:
122:
118:
114:
110:
106:
102:
98:
94:
90:
86:
82:
75:
73:pronunciation
66:
58:
55:
53:
49:
45:
41:
36:
31:
19:
1500:Ether lipids
1401:-serine (PS)
973:Butyrylation
935:
676:
547:Racemization
532:Peptide bond
445:. Retrieved
441:the original
436:
426:
399:
395:
385:
350:
347:Biochemistry
346:
336:
319:
309:
297:
291:
285:
279:
261:
251:
229:
193:
181:
172:. Since the
146:
113:carbohydrate
84:
80:
64:
63:
1561:Metabolites
1543:Sphingosine
1529:Cardiolipin
1509:Plasmalogen
1037:Diphthamide
996:Methylation
963:Lactylation
925:Deamination
915:Sumoylation
890:Acetylation
885:Methylation
844:Deamidation
816:Methylation
765:Prenylation
590:Methylation
580:Formylation
570:Acetylation
542:Proteolysis
215:lipid rafts
133:fatty acids
117:glucosamine
38:Identifiers
1632:Categories
1613:Citicoline
1160:Tryptophan
1156:Tryptophan
1112:Methionine
1053:Tryptophan
836:Asparagine
606:C terminus
562:N terminus
447:2013-07-29
328:References
318:protozoan
242:(DAF) and
207:intestinal
174:glypiation
157:translocon
93:C-terminus
52:Membranome
18:Gpi anchor
1306:Desmosine
1219:Histidine
1039:formation
1029:Histidine
948:Glycation
895:Acylation
859:Glutamine
798:Glutamate
775:Aspartate
717:Sulfation
653:Threonine
614:Amidation
585:Glycation
314:from the
226:In humans
143:Synthesis
1577:Inositol
1545:backbone
1477:PI(4,5)P
1469:PI(3,5)P
1461:PI(3,4)P
1366:backbone
1364:Glycerol
1294:Allysine
1290:Allysine
1286:Allysine
1223:Tyrosine
1196:Tyrosine
1138:Tyrosine
1089:Cysteine
1085:Cysteine
983:Arginine
752:Cysteine
694:Tyrosine
418:23733340
377:18557633
190:Cleavage
183:in vitro
125:inositol
1603:Choline
1254:Glycine
1246:Alanine
1227:Glycine
1200:Glycine
1011:Proline
679:-GlcNAc
525:General
368:2663890
121:mannose
99:during
97:protein
87:) is a
1448:PI(5)P
1443:PI(4)P
1438:PI(3)P
1416:glyco-
1352:Lipids
1298:Lysine
1250:Serine
1192:Serine
1134:Lysine
877:Lysine
649:Serine
471:(MeSH)
416:
375:
365:
199:T-cell
43:Symbol
1418:(GPI)
597:(Gly)
396:Blood
299:PGAP3
293:PGAP2
95:of a
79:) or
1434:PIP
516:and
414:PMID
373:PMID
310:The
296:and
287:PIGO
281:PIGV
272:lyse
270:can
263:PIGT
253:PIGA
244:CD59
209:and
119:and
1487:PIP
1454:PIP
1277:AAs
1183:AAs
1076:AAs
640:AAs
404:doi
400:122
363:PMC
355:doi
246:in
85:GPI
57:327
46:GPI
1634::
1354::
435:.
412:.
398:.
394:.
371:.
361:.
351:47
349:.
345:.
302:.
290:,
284:,
139:.
1502::
1489:3
1479:2
1471:2
1463:2
1456:2
1384::
1374:)
1368:(
1344:e
1337:t
1330:v
1296:–
1292:–
1288:–
1252:–
1248:–
1225:–
1221:–
1198:–
1194:–
1158:–
1136:–
1114:–
1087:–
936:O
677:O
651:/
506:e
499:t
492:v
450:.
420:.
406::
379:.
357::
83:(
67:(
20:)
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