377:
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104:
383:
The ligand (black) when bound in the active site of the enzyme (blue). The two key amino acid residues in the active site are Asp-170 and Asp-231. First, Asp-170 performs a nucleophilic attack on the glycosidic bond to release the terminal α-galactose molecule from the ligand. Then, Asp-231 serves as
779:
772:
765:
61:
269:
288:
360:
This enzyme is a homodimeric glycoprotein that hydrolyses the terminal α-galactosyl moieties from glycolipids and glycoproteins. It predominantly hydrolyzes
1129:
671:
384:
an acid to remove a proton from water, making it more nucleophilic to attack the galactose-Asp complex and release α-galactose from the active site.
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1839:
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1393:
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31:
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It catalyzes many catabolic processes, including cleavage of glycoproteins, glycolipids, and polysaccharides.
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Scriver CR, Sly WS, Childs B, ABeaudet AL, Valle D, Kinzler KW, et al. (15 December 2000).
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939:
450:
Calhoun DH, Bishop DF, Bernstein HS, Quinn M, Hantzopoulos P, Desnick RJ (November 1985).
601:
Vocadlo DJ, Davies GJ (October 2008). "Mechanistic insights into glycosidase chemistry".
165:
467:
124:
30:
This article is about the enzyme. For the carbohydrate commonly called "alpha gal", see
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339:-galactosides, including galactose oligosaccharides, galactomannans and galactolipids
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17:
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Proceedings of the
National Academy of Sciences of the United States of America
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452:"Fabry disease: isolation of a cDNA clone encoding human α-galactosidase A"
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381:
A double displacement reaction mechanism of α-GAL's catalytic action.
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554:: purification and characterization of the main α-galactosidase"
184:
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1111:
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103:
681:
36:
1489:
511:"Stereochemistry and the Mechanism of Enzymatic Reactions"
427:
The
Metabolic & Molecular Basis of Inherited Disease
57:
406:
Classification of α-galactosidases (according to CAZy)
1780:
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1640:
1609:
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1204:
1163:
1150:
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1029:
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953:
839:
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364:trihexoside, and it can catalyze the hydrolysis of
287:
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142:
130:
118:
113:
96:
52:
may be too technical for most readers to understand
1505:
1123:
773:
697:
108:α-Galactosidase tetramer, Mortierella vinacea
8:
548:Brumer H, Sims PF, Sinnott ML (April 1999).
1512:
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672:United States National Library of Medicine
255:
642:at the U.S. National Library of Medicine
577:
485:
475:
80:Learn how and when to remove this message
64:, without removing the technical details.
747:: Structure of human alpha-galactosidase
732:: Structure of human alpha-galactosidase
670:This article incorporates text from the
1787:
719:
417:
331:Hydrolysis of terminal, non-reducing α-
327:that catalyses the following reaction:
93:
62:make it understandable to non-experts
7:
603:Current Opinion in Chemical Biology
527:10.1111/j.1469-185x.1953.tb01386.x
402:, a drug targeting α-galactosidase
25:
923:Sphingomyelin phosphodiesterase 1
1802:
1790:
737:
722:
102:
41:
1394:Alpha-N-acetylgalactosaminidase
918:Sphingomyelin phosphodiesterase
550:"Lignocellulose degradation by
1039:Palmitoyl protein thioesterase
1:
1406:Alpha-N-acetylglucosaminidase
1082:Serine C-palmitoyltransferase
429:(8th ed.). McGraw-Hill.
346:The enzyme is encoded by the
318:-galactoside galactohydrolase
32:Galactose-alpha-1,3-galactose
1092:Ceramide glucosyltransferase
368:into galactose and glucose.
552:Phanerochaete chrysosporium
1856:
1840:Enzymes of known structure
661:gene details page in the
615:10.1016/j.cbpa.2008.05.010
29:
1668:Michaelis–Menten kinetics
717:
335:-galactose residues in α-
254:
101:
1560:Diffusion-limited enzyme
644:Medical Subject Headings
1316:Bacterial neuraminidase
1044:Tripeptidyl peptidase I
558:The Biochemical Journal
477:10.1073/pnas.82.21.7364
1479:Oxoguanine glycosylase
385:
1653:Eadie–Hofstee diagram
1586:Allosteric regulation
379:
1663:Lineweaver–Burk plot
1468:N-Glycosyl compounds
1438:Maltase-glucoamylase
1389:Galactosylceramidase
1156:Glycoside hydrolases
1141:: sugar hydrolases (
945:Galactosylceramidase
655:genome location and
509:Koshland DE (1953).
1321:Viral neuraminidase
893:Alpha-galactosidase
826:Glycosyltransferase
663:UCSC Genome Browser
640:alpha-Galactosidase
468:1985PNAS...82.7364C
322:glycoside hydrolase
18:Alpha galactosidase
1622:Enzyme superfamily
1555:Enzyme promiscuity
1372:Glucosylceramidase
1253:Debranching enzyme
1177:Sucrase-isomaltase
1074:Ceramide synthesis
1017:Sphingosine kinase
903:Glucocerebrosidase
898:Beta-galactosidase
873:Glucocerebrosidase
858:Beta-galactosidase
674:, which is in the
515:Biological Reviews
386:
372:Reaction mechanism
312:; systematic name
1778:
1777:
1487:
1486:
1458:
1457:
1345:alpha-Mannosidase
1219:Alpha-glucosidase
1105:
1104:
1025:
1024:
818:glycosphingolipid
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754:
570:10.1042/bj3390043
436:978-0-07-913035-8
338:
334:
317:
303:
302:
299:
298:
202:metabolic pathway
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16:(Redirected from
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1807:
1806:
1805:
1795:
1794:
1786:
1658:Hanes–Woolf plot
1601:Enzyme activator
1596:Enzyme inhibitor
1570:Enzyme catalysis
1514:
1507:
1500:
1491:
1475:DNA glycosylases
1241:Beta-glucosidase
1161:
1132:
1125:
1118:
1109:
936:
914:
888:Hexosaminidase B
884:
863:Hexosaminidase A
854:
831:Sulfotransferase
813:
793:lipid metabolism
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1686:Oxidoreductases
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1648:Enzyme kinetics
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1632:List of enzymes
1605:
1574:
1545:Catalytic triad
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1454:
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1200:
1146:
1136:
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1101:
1068:
1021:
1005:
949:
940:Arylsulfatase A
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849:
835:
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636:
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395:β-galactosidase
391:
374:
358:
308:( EC 3.2.1.22,
306:α-Galactosidase
109:
97:α-galactosidase
86:
75:
69:
66:
58:help improve it
55:
46:
42:
35:
28:
23:
22:
15:
12:
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1610:Classification
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1416:Hexosaminidase
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1328:Galactosidases
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1210:
1208:
1202:
1201:
1199:
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1183:
1169:
1167:
1165:Disaccharidase
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634:External links
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521:(4): 416–436.
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462:(21): 7364–8.
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310:α-GAL, α-GAL A
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1627:Enzyme family
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1592:
1591:Cooperativity
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1550:Oxyanion hole
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1470:
1466:: Hydrolysing
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1431:
1427:
1424:
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1404:
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1390:
1387:
1383:
1382:non-lysosomal
1380:
1378:
1375:
1374:
1373:
1370:
1368:
1365:
1363:
1362:Hyaluronidase
1360:
1356:
1353:
1352:
1351:
1350:Glucuronidase
1348:
1346:
1343:
1339:
1336:
1334:
1331:
1330:
1329:
1326:
1322:
1319:
1317:
1314:
1312:
1309:
1307:
1304:
1302:
1299:
1297:
1294:
1293:
1292:
1291:Neuraminidase
1289:
1287:
1284:
1282:
1279:
1275:
1274:Alpha-amylase
1272:
1271:
1270:
1267:
1266:
1264:
1260:
1254:
1251:
1247:
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1243:
1242:
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1221:
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1212:
1211:
1209:
1207:
1203:
1197:
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1192:
1189:
1187:
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1182:
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1174:
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1168:
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1157:
1153:
1149:
1144:
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1133:
1128:
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1119:
1114:
1113:
1110:
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1080:
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912:sphingomyelin
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901:
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871:
869:
868:Neuraminidase
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676:public domain
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609:(5): 539–55.
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240:Gene Ontology
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178:NiceZyme view
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50:This article
48:
39:
38:
33:
19:
1764:Translocases
1761:
1748:
1735:
1722:
1709:
1699:Transferases
1696:
1683:
1540:Binding site
1206:Glucosidases
808:Sphingolipid
744:
729:
669:
668:
657:
651:
606:
602:
596:
564:(1): 43–53.
561:
557:
551:
543:
518:
514:
504:
459:
455:
445:
426:
420:
380:
359:
347:
345:
342:
313:
309:
305:
304:
166:BRENDA entry
91:
76:
70:October 2022
67:
51:
1535:Active site
1433:Iduronidase
1367:Pullulanase
956:sphingosine
852:ganglioside
712:PDB gallery
154:IntEnz view
114:Identifiers
1819:Categories
1738:Isomerases
1712:Hydrolases
1579:Regulation
1443:Heparanase
1411:Fucosidase
1229:Neutral AB
964:Ceramidase
797:glycolipid
789:Metabolism
412:References
400:Migalastat
223:structures
190:KEGG entry
137:9025-35-8
1617:EC number
1377:lysosomal
1281:Chitinase
1246:cytosolic
1234:Neutral C
1214:Cellulase
1191:Trehalase
1181:Invertase
1139:Hydrolase
934:sulfatide
882:globoside
366:melibiose
143:Databases
1825:EC 3.2.1
1809:Medicine
1641:Kinetics
1565:Cofactor
1528:Activity
1286:Lysozyme
842:ceramide
623:18558099
588:10085226
535:86709302
389:See also
362:ceramide
356:Function
294:proteins
282:articles
270:articles
227:RCSB PDB
125:3.2.1.22
1830:Enzymes
1797:Biology
1783:Portals
1751:Ligases
1521:Enzymes
1269:Amylase
1196:Lactase
1186:Maltase
1173:Sucrase
800:enzymes
579:1220126
496:2997789
464:Bibcode
320:) is a
249:QuickGO
214:profile
197:MetaCyc
132:CAS no.
56:Please
1835:Sanofi
1725:Lyases
1355:Klotho
1086:SPTLC1
999:ASAH2C
994:ASAH2B
649:Human
646:(MeSH)
621:
586:
576:
533:
494:
487:391345
484:
433:
352:gene.
325:enzyme
277:PubMed
259:Search
245:AmiGO
233:PDBsum
173:ExPASy
161:BRENDA
149:IntEnz
120:EC no.
27:Enzyme
1677:Types
1464:3.2.2
1448:HPSE2
1333:Alpha
1262:Other
1152:3.2.1
1010:Other
989:ASAH2
984:ASAH1
979:ACER3
974:ACER2
969:ACER1
932:From
910:From
880:From
850:From
531:S2CID
209:PRIAM
1769:list
1762:EC7
1756:list
1749:EC6
1743:list
1736:EC5
1730:list
1723:EC4
1717:list
1710:EC3
1704:list
1697:EC2
1691:list
1684:EC1
1426:HEXB
1421:HEXA
1399:NAGA
1338:Beta
1311:NEU4
1306:NEU3
1301:NEU2
1296:NEU1
1224:Acid
1145:3.2)
1096:UGCG
1064:CLN8
1059:CLN6
1054:CLN5
1049:CLN3
745:1r47
730:1r46
619:PMID
584:PMID
492:PMID
431:ISBN
289:NCBI
230:PDBe
185:KEGG
1031:NCL
954:To
840:To
816:To
658:GLA
652:GLA
611:doi
574:PMC
566:doi
562:339
523:doi
482:PMC
472:doi
349:GLA
265:PMC
221:PDB
60:to
1821::
1477::
1154::
1143:EC
795:,
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617:.
607:12
605:.
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458:.
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314:α-
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337:D
333:D
316:D
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