31:
1703:, treX, provides an interesting example of using a single active site for two activities: amylosidase and glucanotransferase activities. TreX is structurally similar to glgX, and has a mass of 80kD and one active site. Unlike either glgX, however, treX exists as a dimer and tetramer in solution. TreX's oligomeric form seems to play a significant role in altering both enzyme shape and function. Dimerization is thought to stabilize a "flexible loop" located close to the active site. This may be key to explaining why treX (and not glgX) shows glucosyltransferase activity. As a tetramer, the catalytic efficiency of treX is increased fourfold over its dimeric form.
1715:, a single enzyme performs both debranching functions. The human glycogen debranching enzyme (gene: AGL) is a monomer with a molecular weight of 175 kDa. It has been shown that the two catalytic actions of AGL can function independently of each other, demonstrating that multiple active sites are present. This idea has been reinforced with inhibitors of the active site, such as polyhydroxyamine, which were found to inhibit glucosidase activity while transferase activity was not measurably changed. Glycogen debranching enzyme is the only known eukaryotic enzyme that contains multiple catalytic sites and is active as a monomer.
249:
226:
123:
148:
1175:, in the mobilization of glycogen stores. Phosphorylase can only cleave α-1,4-glycosidic bond between adjacent glucose molecules in glycogen but branches also exist as α-1,6 linkages. When phosphorylase reaches four residues from a branching point it stops cleaving; because 1 in 10 residues is branched, cleavage by phosphorylase alone would not be sufficient in mobilizing glycogen stores. Before phosphorylase can resume catabolism, debranching enzymes perform two functions:
255:
154:
1199:
4436:
1673:, Glucose transfer is performed by 4-alpha-glucanotransferase, a 78.5 kDa protein coded for by the gene malQ. A second protein, referred to as debranching enzyme, performs α-1,6-glucose cleavage. This enzyme has a molecular mass of 73.6 kDa, and is coded for by the gene glgX. Activity of the two enzymes is not always necessarily coupled. In
1802:. Type IIIa patients often exhibit symptoms related to liver disease and progressive muscle involvement, with variations caused by age of onset, rate of disease progression and severity. Patients with Type IIIb generally symptoms related to liver disease. Type III patients be distinguished by elevated liver enzymes, with normal
1727:
1691:. The monomeric protein contains a central domain in which eight parallel beta-strands are surrounded by eight parallel alpha strands. Notable within this structure is a groove 26 angstroms long and 9 angstroms wide, containing aromatic residues that are thought to stabilize a four-glucose branch before cleavage.
1753:
department of pediatrics at Duke
University suggest that the human AGL gene contains at minimum 2 promotor regions, sites where the transcription of the gene begins, that result in differential expression of isoform, different forms of the same protein, mRNAs in a manner that is specific for different tissues.
1738:
GDE has been reported. The structure revealed that distinct domains in GDE encode the glucanotransferase and glucosidase activities. Their catalyses are similar to that of alpha-amylase and glucoamylase, respectively. Their active sites are selective towards the respective substrates, ensuring proper
1747:
The official name for the gene is "amylo-α-1,6-glucosidase, 4-α-glucanotransferase", with the official symbol AGL. AGL is an autosomal gene found on chromosome 1p21. The AGL gene provides instructions for making several different versions, known as isoforms, of the glycogen debranching enzyme. These
1767:
When GDE activity is compromised, the body cannot effectively release stored glycogen, type III Glycogen
Storage Disease (debrancher deficiency), an autosomal recessive disorder, can result. In GSD III glycogen breakdown is incomplete and there is accumulation of abnormal glycogen with short outer
1752:
and encodes for a 7.0 kb mRNA. Translation of the gene begins at exon 3,which encodes for the first 27 amino acids of the AGL gene, because the first two exons (68kb) contain the 5' untranslated region. Exons 4-35 encode the remaining 1505 amino acids of the AGL gene. Studies produced by the
1723:, AGL appears to contain a third active site that allows it to bind to a glycogen polymer. It is thought to bind to six glucose molecules of the chain as well as the branched glucose, thus corresponding to 7 subunits within the active site, as shown in the figure below.
1739:
activation of GDE. Besides the active sites GDE have additional binding sites for glycogen, which are important for its recruitment to glycogen. Mapping the disease-causing mutations onto the GDE structure provided insights into glycogen storage disease type III.
1748:
isoforms vary by size and are expressed in different tissues, such as liver and muscle. This gene has been studied in great detail, because mutation at this gene is the cause of
Glycogen Storage Disease Type III. The gene is 85 kb long, has 35
1170:
and glucose mobilization. When phosphorylase has digested a glycogen branch down to four glucose residues, it will not remove further residues. Glycogen debranching enzymes assist phosphorylase, the primary enzyme involved in
2146:
Hondoh H, Saburi W, Mori H, Okuyama M, Nakada T, Matsuura Y, et al. (May 2008). "Substrate recognition mechanism of alpha-1,6-glucosidic linkage hydrolyzing enzyme, dextran glucosidase from
Streptococcus mutans".
2527:
Gillard BK, Nelson TE (September 1977). "Amylo-1,6-glucosidase/4-alpha-glucanotransferase: use of reversible substrate model inhibitors to study the binding and active sites of rabbit muscle debranching enzyme".
2897:
1718:
Some studies have shown that the C-terminal half of yeast GDE is associated with glucosidase activity, while the N-terminal half is associated with glucosyltransferase activity. In addition to these two
3581:
3576:
2563:
Yamamoto E, Makino Y, Omichi K (May 2007). "Active site mapping of amylo-alpha-1,6-glucosidase in porcine liver glycogen debranching enzyme using fluorogenic 6-O-alpha-glucosyl-maltooligosaccharides".
2430:"Amylo-1,6-glucosidase/4-alpha-glucanotransferase. Reaction of rabbit muscle debranching enzyme with an active site-directed irreversible inhibitor, 1-S-dimethylarsino-1-thio-beta-D-glucopyranoside"
1109:
reserves from glycogen deposits in the muscles and liver. This constitutes a major source of energy reserves in most organisms. Glycogen breakdown is highly regulated in the body, especially in the
1806:
and blood lactate levels, differing from other forms of GSD. In patients with muscle involvement, Type IIIa, the muscle weakness becomes predominant into adulthood and can lead to ventricular
1771:
Most patients exhibit GDE defiency in both liver and muscle (Type IIIa), although 15% of patients have retained GDE in muscle while having it absent from the liver (Type IIIb). Depending on
1216:, cleaves the remaining alpha-1,6 linkage, producing glucose and a linear chain of glycogen. The mechanism by which the glucosidase cleaves the α -1,6-linkage is not fully known because the
1937:
Song HN, Jung TY, Park JT, Park BC, Myung PK, Boos W, et al. (June 2010). "Structural rationale for the short branched substrate specificity of the glycogen debranching enzyme GlgX".
2349:
Park JT, Park HS, Kang HK, Hong JS, Cha H, Woo EJ, et al. (2008). "Oligomeric and functional properties of a debranching enzyme (TreX) from the archaeon
Sulfobus solfataricus P2".
3954:
3830:
2655:
Ding JH, de Barsy T, Brown BI, Coleman RA, Chen YT (January 1990). "Immunoblot analyses of glycogen debranching enzyme in different subtypes of glycogen storage disease type III".
2890:
1240:
Thus the debranching enzymes, transferase and α-1,6-glucosidase converts the branched glycogen structure into a linear one, paving the way for further cleavage by phosphorylase.
3621:
1121:, to maintain a homeostatic balance of blood-glucose levels. When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as
1987:
Bao Y, Dawson TL, Chen YT (December 1996). "Human glycogen debranching enzyme gene (AGL): complete structural organization and characterization of the 5' flanking region".
2722:"Mutations in exon 3 of the glycogen debranching enzyme gene are associated with glycogen storage disease type III that is differentially expressed in liver and muscle"
2883:
262:
161:
2940:
1613:
1412:
3935:
4003:
3452:
1632:
1431:
1194:
from the four-residue glycogen branch to a nearby branch. This exposes a single glucose residue joined to the glucose chain through an α-1,6 glycosidic linkage
4112:
4082:
3070:
784:
3757:
3752:
765:
84:
1228:
ion intermediate, and retention of configuration in glucose. This is a common method through which to cleave bonds, with an acid below the site of
4117:
30:
2699:
2125:
1886:
4456:
1868:
1677:
glgX selectively catalyzes the cleavage of 4-subunit branches, without the action of glucanotransferase. The product of this cleavage,
1236:. These acids and bases are amino acid side chains in the active site of the enzyme. A scheme for the mechanism is shown in the figure.
248:
3445:
2062:"Structural insight into the bifunctional mechanism of the glycogen-debranching enzyme TreX from the archaeon Sulfolobus solfataricus"
1027:
1020:
4155:
3998:
2471:"Glycogen debranching enzyme: purification, antibody characterization, and immunoblot analyses of type III glycogen storage disease"
1762:
1122:
225:
2769:
Talente GM, Coleman RA, Alter C, Baker L, Brown BI, Cannon RA, et al. (February 1994). "Glycogen storage disease in adults".
4461:
4008:
1625:
1424:
4029:
3334:
2875:
1855:
1834:
4013:
3063:
1576:
1552:
1375:
1351:
4311:
1851:
147:
122:
3438:
3346:
4426:
1830:
3548:
1786:
These different manifestation produce varied symptoms, which can be nearly indistinguishable from Type I GSD, including
64:
1224:
have not yet been identified. It is thought to proceed through a two step acid base assistance type mechanism, with an
3973:
1250:
261:
160:
4296:
1098:, which serves as a store of glucose in the body. It has separate glucosyltransferase and glucosidase activities.
4412:
4399:
4386:
4373:
4360:
4347:
4334:
4098:
3990:
3903:
3538:
3478:
3404:
3092:
3056:
2950:
4306:
2600:"Crystal structure of glycogen debranching enzyme and insights into its catalysis and disease-causing mutations"
1726:
1570:
1369:
254:
153:
4260:
4203:
3543:
3516:
3469:
3083:
2869:
1463:
1262:
1206:
1180:
829:
72:
1669:
and other bacteria, glucosyltransferase and glucosidase functions are performed by two distinct proteins. In
1557:
1356:
4208:
4033:
3596:
3256:
2910:
1699:
810:
3511:
3419:
3164:
3011:
2978:
2855:
1637:
1436:
4229:
4148:
4077:
3563:
3553:
1545:
1344:
1138:
and other bacteria, complicating nomenclature. Proteins that catalyze both functions are referred to as
4301:
2865:
3945:
3772:
3506:
3378:
3329:
3032:
2611:
1480:
1279:
136:
2286:
Dauvillée D, Kinderf IS, Li Z, Kosar-Hashemi B, Samuel MS, Rampling L, et al. (February 2005).
1128:
The two steps of glycogen breakdown, glucosyltransferase and glucosidase, are performed by a single
4265:
3907:
3822:
3720:
3493:
3465:
3273:
3261:
3096:
3037:
1573:
1475:
1372:
1274:
1191:
1187:
942:
938:
51:
1497:
1296:
4198:
4060:
3744:
3482:
3317:
3312:
3290:
3278:
3117:
2988:
2935:
2794:
2366:
1962:
96:
2860:
984:
980:
976:
972:
968:
917:
907:
903:
899:
895:
891:
4471:
4466:
4102:
3528:
3430:
3285:
3159:
3007:
2945:
2835:
2786:
2751:
2695:
2672:
2637:
2580:
2545:
2492:
2451:
2410:
2317:
2240:
2205:
2164:
2121:
2093:
2039:
2004:
1954:
1564:
1363:
44:
4244:
4239:
4213:
4141:
3940:
3927:
3571:
3521:
3295:
3181:
2825:
2782:
2778:
2741:
2733:
2664:
2627:
2619:
2572:
2537:
2482:
2441:
2400:
2358:
2307:
2299:
2232:
2195:
2156:
2083:
2073:
2031:
2022:
Hers HG, Verhue W, Van hoof F (October 1967). "The determination of amylo-1,6-glucosidase".
1996:
1946:
1665:
341:
272:
216:
171:
3812:
3807:
1533:
1332:
4291:
4275:
4188:
3959:
3415:
1776:
316:
2223:
McCarter JD, Withers SG (December 1994). "Mechanisms of enzymatic glycoside hydrolysis".
1509:
1308:
92:
2615:
1468:
1267:
4440:
4329:
4270:
3356:
3268:
3105:
3048:
2918:
2632:
2599:
2487:
2470:
2088:
2061:
2035:
1799:
1608:
1407:
1172:
1167:
1091:
2746:
2721:
2668:
2446:
2429:
2389:"Identification of the catalytic residues of bifunctional glycogen debranching enzyme"
2312:
2287:
1588:
1387:
1198:
699:
694:
689:
684:
679:
674:
669:
664:
648:
643:
638:
633:
628:
623:
618:
613:
608:
592:
587:
582:
577:
572:
567:
562:
557:
547:
542:
537:
532:
4450:
4234:
4193:
3964:
3501:
3302:
3231:
3214:
2236:
1678:
1583:
1382:
1163:
1102:
519:
2798:
2370:
2303:
1966:
1142:(GDEs). When glucosyltransferase and glucosidase are catalyzed by distinct enzymes,
76:
4183:
3146:
2914:
2812:
Kishnani PS, Austin SL, Arn P, Bali DS, Boney A, Case LE, et al. (July 2010).
1791:
1787:
1775:
location, different mutations in the AGL gene can affect different isoforms of the
1225:
334:
113:
2830:
2813:
2273:"Glycogen debranching enzyme - Escherichia coli O139:H28 (strain E24377A / ETEC)"
1232:
to lend a proton and a base above to deprotinate a water which can then act as a
100:
4407:
4342:
4178:
3461:
3373:
3339:
3307:
1891:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1873:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1807:
1779:. For example, mutations that occur on exon 3, affect the form which affect the
1720:
1592:
1391:
1233:
1221:
1217:
1213:
1150:. In some literature, an enzyme capable only of glucosidase is referred to as a
4435:
417:
3383:
3351:
2955:
2906:
2362:
2160:
1908:"Genes (Genetic Home Reference a service of U.S. National Library of Medicine"
1688:
1229:
233:
130:
80:
2060:
Woo EJ, Lee S, Cha H, Park JT, Yoon SM, Song HN, et al. (October 2008).
4381:
4355:
3221:
3154:
3131:
3121:
3079:
1803:
729:
477:
355:
300:
287:
199:
186:
88:
2839:
2641:
2584:
2414:
2405:
2388:
2321:
2168:
2097:
2078:
2000:
1958:
1783:
that is primarily expressed in the liver; this would lead to GSD type III.
2790:
2755:
2676:
2496:
2455:
2244:
2209:
2043:
2008:
1067:
1062:
3226:
2998:
2549:
2510:
2335:
2272:
2258:
1795:
1772:
1118:
1095:
1051:
855:
2623:
2576:
2541:
2200:
2183:
1521:
1320:
1209:
1183:
552:
4122:
3980:
3713:
3708:
3703:
3698:
3693:
3661:
3585:
3209:
3136:
3126:
3113:
2814:"Glycogen storage disease type III diagnosis and management guidelines"
1950:
1780:
1695:
1540:
1339:
1134:
1114:
1106:
841:
796:
2737:
4394:
4164:
3918:
3688:
3683:
3678:
3673:
3668:
3656:
3651:
3646:
3641:
3636:
3631:
3626:
3614:
3609:
3604:
2921:
1620:
1516:
1504:
1492:
1419:
1315:
1303:
1291:
1147:
1132:
in mammals, yeast, and some bacteria, but by two distinct enzymes in
1129:
1035:
751:
2692:
Molecular
Pathology of Liver Diseases (Molecular Pathology Library)
4368:
4070:
4065:
4038:
3889:
3840:
3835:
3388:
3169:
2856:
GeneReviews/NCBI/NIH/UW entry on
Glycogen Storage Disease Type III
1907:
1725:
1712:
1197:
1110:
714:
710:
3884:
3879:
3874:
3869:
3864:
3859:
3852:
3847:
3802:
3797:
3792:
3787:
3782:
3777:
3767:
3762:
3734:
3729:
3724:
3366:
3361:
3322:
3251:
3246:
3241:
3236:
3186:
3174:
1749:
1528:
1327:
1084:
68:
4137:
3582:
Glycoprotein-N-acetylgalactosamine 3-beta-galactosyltransferase
3577:
B-N-acetylglucosaminyl-glycopeptide b-1,4-galactosyltransferase
3434:
3052:
2879:
2720:
Shen J, Bao Y, Liu HM, Lee P, Leonard JV, Chen YT (July 1996).
2288:"Role of the Escherichia coli glgX gene in glycogen metabolism"
55:, GDE, amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase
2428:
Gillard BK, White RC, Zingaro RA, Nelson TE (September 1980).
2259:"4-alpha-glucanotransferase - Escherichia coli (strain K12)"
2184:"Molecular mechanism in alpha-glucosidase and glucoamylase"
4133:
324:
3831:
Dolichyl-phosphate-mannose-protein mannosyltransferase
4424:
1681:, is further degraded by maltodextrin phosphorylase.
489:
2598:
Zhai L, Feng L, Xia L, Yin H, Xiang S (April 2016).
2511:"Glycogen debranching enzyme - Homo sapiens (Human)"
588:
polyubiquitin modification-dependent protein binding
4320:
4284:
4253:
4222:
4171:
4097:
4053:
4022:
3989:
3926:
3917:
3902:
3821:
3743:
3595:
3562:
3492:
3477:
3403:
3202:
3145:
3104:
3091:
3025:
2997:
2971:
2964:
2928:
2469:Chen YT, He JK, Ding JH, Brown BI (December 1987).
1631:
1619:
1607:
1602:
1582:
1563:
1551:
1539:
1527:
1515:
1503:
1491:
1486:
1474:
1462:
1457:
1452:
1430:
1418:
1406:
1401:
1381:
1362:
1350:
1338:
1326:
1314:
1302:
1290:
1285:
1273:
1261:
1256:
1249:
998:
961:
931:
890:
1847:
1845:
1843:
1826:
1824:
1822:
2861:OMIM entries on Glycogen Storage Disease Type III
271:
170:
2387:Nakayama A, Yamamoto K, Tabata S (August 2001).
593:beta-maltose 4-alpha-glucanotransferase activity
4004:Hypoxanthine-guanine phosphoribosyltransferase
1852:GRCm38: Ensembl release 89: ENSMUSG00000033400
4149:
3446:
3064:
2891:
2120:(6th ed.). San Francisco: W.H. Freeman.
8:
4113:Beta-galactoside alpha-2,6-sialyltransferase
1687:GlgX is structurally similar to the protein
1202:Mechanism for cleaving of alpha-1,6 linkage.
543:hydrolase activity, acting on glycosyl bonds
4083:Indolylacetylinositol arabinosyltransferase
2382:
2380:
2188:Bioscience, Biotechnology, and Biochemistry
1831:GRCh38: Ensembl release 89: ENSG00000162688
1166:, glycogen debranching enzymes function in
1083:, in humans, is the protein encoded by the
4156:
4142:
4134:
3923:
3914:
3489:
3453:
3439:
3431:
3101:
3071:
3057:
3049:
2968:
2898:
2884:
2876:
2522:
2520:
1599:
1398:
887:
725:
515:
374:Skeletal muscle tissue of rectus abdominis
312:
211:
108:
2868:at the U.S. National Library of Medicine
2829:
2745:
2631:
2486:
2445:
2404:
2311:
2199:
2141:
2139:
2137:
2087:
2077:
1242:
378:Skeletal muscle tissue of biceps brachii
4431:
2783:10.7326/0003-4819-120-3-199402010-00008
2715:
2713:
2711:
2116:Stryer L, Berg JM, Tymoczko JL (2007).
2111:
2109:
2107:
1818:
16:Mammalian protein found in Homo sapiens
4118:Monosialoganglioside sialyltransferase
3955:NAD(P):arginine ADP-ribosyltransferase
3936:NAD:diphthamide ADP-ribosyltransferase
1932:
1930:
1928:
1926:
1924:
1902:
1900:
1449:
1246:
20:
2726:The Journal of Clinical Investigation
2225:Current Opinion in Structural Biology
2055:
2053:
1982:
1980:
1978:
1976:
1694:The glycogen-degrading enzyme of the
276:
237:
232:
175:
134:
129:
7:
1730:Hypothesized sidechain binding sites
573:glycogen debranching enzyme activity
568:amylo-alpha-1,6-glucosidase activity
2434:The Journal of Biological Chemistry
2393:The Journal of Biological Chemistry
2066:The Journal of Biological Chemistry
1707:One enzyme with two catalytic sites
1090:. This enzyme is essential for the
563:4-alpha-glucanotransferase activity
452:medial head of gastrocnemius muscle
2475:American Journal of Human Genetics
2351:Biocatalysis and Biotransformation
2336:"TreX - Actinoplanes sp. SN223/29"
2036:10.1111/j.1432-1033.1967.tb00133.x
1146:usually refers to the glucosidase
995:
958:
928:
865:
846:
820:
801:
775:
756:
494:
412:
350:
329:
14:
3999:Adenine phosphoribosyltransferase
1763:Glycogen storage disease type III
1123:Glycogen storage disease type III
4434:
4009:Uracil phosphoribosyltransferase
2024:European Journal of Biochemistry
1113:, by various hormones including
260:
253:
247:
224:
159:
152:
146:
121:
29:
4030:Purine nucleoside phosphorylase
3335:Alpha-N-acetylgalactosaminidase
2304:10.1128/JB.187.4.1465-1473.2005
4014:Amidophosphoribosyltransferase
478:More reference expression data
1:
3347:Alpha-N-acetylglucosaminidase
2941:UDP-glucose pyrophosphorylase
2669:10.1016/S0022-3476(05)81652-X
2447:10.1016/S0021-9258(18)43517-X
665:glycogen biosynthetic process
245:
144:
3549:Ceramide glucosyltransferase
2831:10.1097/GIM.0b013e3181e655b6
2237:10.1016/0959-440X(94)90271-2
2149:Journal of Molecular Biology
1794:in children, short stature,
1140:glycogen debranching enzymes
634:ficolin-1-rich granule lumen
538:glycosyltransferase activity
4457:Genes on human chromosome 1
2866:Glycogen+debranching+enzyme
2771:Annals of Internal Medicine
1810:and distal muscle wasting.
1144:glycogen debranching enzyme
1081:glycogen debranching enzyme
4488:
3974:Poly ADP ribose polymerase
1760:
1190:, transfers three glucose
1179:4-α-D-glucanotransferase (
700:response to glucocorticoid
685:glycogen metabolic process
670:glycogen catabolic process
440:sternocleidomastoid muscle
4312:Michaelis–Menten kinetics
3991:Phosphoribosyltransferase
2951:Glycogen branching enzyme
2657:The Journal of Pediatrics
2363:10.1080/10242420701806652
2161:10.1016/j.jmb.2008.03.016
1887:"Mouse PubMed Reference:"
1869:"Human PubMed Reference:"
1598:
1397:
1205:Amylo-α-1,6-glucosidase (
1066:
1061:
1057:
1050:
1034:
1015:
1002:
965:
954:
935:
914:
883:
872:
868:
853:
849:
840:
827:
823:
808:
804:
795:
782:
778:
763:
759:
750:
735:
728:
724:
708:
518:
514:
502:
497:
488:
475:
424:
415:
362:
353:
323:
315:
311:
294:
281:
244:
223:
214:
210:
193:
180:
143:
120:
111:
107:
62:
59:
49:
42:
37:
28:
23:
4204:Diffusion-limited enzyme
3544:1,3-Beta-glucan synthase
2870:Medical Subject Headings
1028:Chr 3: 116.53 – 116.6 Mb
680:neutrophil degranulation
464:tibialis anterior muscle
402:tibialis anterior muscle
4462:Carbohydrate metabolism
4034:Thymidine phosphorylase
3257:Bacterial neuraminidase
2911:carbohydrate metabolism
2565:Journal of Biochemistry
2292:Journal of Bacteriology
2182:Chiba S (August 1997).
1700:Sulfolobus solfataricus
1453:amylo-α-1,6-glucosidase
1021:Chr 1: 99.85 – 99.92 Mb
629:secretory granule lumen
436:vastus lateralis muscle
366:vastus lateralis muscle
3928:ADP-ribosyltransferase
3420:Oxoguanine glycosylase
2979:Glycogen phosphorylase
2406:10.1074/jbc.M102192200
2079:10.1074/jbc.M802560200
2001:10.1006/geno.1996.0611
1731:
1654:Structure and activity
1251:4-α-glucanotransferase
1203:
1105:, the enzyme mobilize
649:sarcoplasmic reticulum
583:polysaccharide binding
432:triceps brachii muscle
390:triceps brachii muscle
4297:Eadie–Hofstee diagram
4230:Allosteric regulation
4078:Arabinosyltransferase
3554:N-glycosyltransferase
2604:Nature Communications
1757:Clinical significance
1734:The structure of the
1729:
1201:
4307:Lineweaver–Burk plot
3946:Pseudomonas exotoxin
3466:glycosyltransferases
3409:N-Glycosyl compounds
3379:Maltase-glucoamylase
3330:Galactosylceramidase
3097:Glycoside hydrolases
3082:: sugar hydrolases (
3033:Phosphorylase kinase
2818:Genetics in Medicine
2694:. Berlin: Springer.
690:response to nutrient
624:extracellular region
578:carbohydrate binding
533:transferase activity
448:gastrocnemius muscle
239:Chromosome 3 (mouse)
137:Chromosome 1 (human)
3721:Hyaluronan synthase
3262:Viral neuraminidase
3038:Protein phosphatase
2624:10.1038/ncomms11229
2616:2016NatCo...711229Z
2542:10.1021/bi00637a007
2399:(31): 28824–28828.
2201:10.1271/bbb.61.1233
2072:(42): 28641–28648.
1188:glucosyltransferase
695:response to hormone
4266:Enzyme superfamily
4199:Enzyme promiscuity
4061:Xylosyltransferase
3534:Debranching enzyme
3313:Glucosylceramidase
3194:Debranching enzyme
3118:Sucrase-isomaltase
2989:Phosphoglucomutase
2984:Debranching enzyme
2936:Phosphoglucomutase
1951:10.1002/prot.22697
1732:
1204:
1173:glycogen breakdown
1168:glycogen breakdown
1152:debranching enzyme
830:ENSMUSG00000033400
658:Biological process
619:isoamylase complex
602:Cellular component
558:hydrolase activity
553:catalytic activity
526:Molecular function
460:intercostal muscle
382:thoracic diaphragm
4422:
4421:
4131:
4130:
4093:
4092:
4049:
4048:
3898:
3897:
3529:Glycogen synthase
3428:
3427:
3399:
3398:
3286:alpha-Mannosidase
3160:Alpha-glucosidase
3046:
3045:
3021:
3020:
3008:Alpha-glucosidase
2946:Glycogen synthase
2738:10.1172/JCI118799
2701:978-1-4419-7106-7
2690:Monga SP (2010).
2577:10.1093/jb/mvm065
2536:(18): 3978–3987.
2440:(18): 8451–8457.
2127:978-0-7167-8724-2
1651:
1650:
1647:
1646:
1643:
1642:
1546:metabolic pathway
1446:
1445:
1442:
1441:
1345:metabolic pathway
1077:
1076:
1073:
1072:
1046:
1045:
1011:
1010:
992:
991:
950:
949:
925:
924:
879:
878:
862:
861:
836:
835:
817:
816:
791:
790:
772:
771:
720:
719:
510:
509:
506:
505:
484:
483:
471:
470:
409:
408:
307:
306:
206:
205:
4479:
4439:
4438:
4430:
4302:Hanes–Woolf plot
4245:Enzyme activator
4240:Enzyme inhibitor
4214:Enzyme catalysis
4158:
4151:
4144:
4135:
3941:Diphtheria toxin
3924:
3915:
3572:Lactose synthase
3539:Branching enzyme
3490:
3455:
3448:
3441:
3432:
3416:DNA glycosylases
3182:Beta-glucosidase
3102:
3073:
3066:
3059:
3050:
2969:
2900:
2893:
2886:
2877:
2844:
2843:
2833:
2809:
2803:
2802:
2766:
2760:
2759:
2749:
2717:
2706:
2705:
2687:
2681:
2680:
2652:
2646:
2645:
2635:
2595:
2589:
2588:
2560:
2554:
2553:
2524:
2515:
2514:
2507:
2501:
2500:
2490:
2481:(6): 1002–1015.
2466:
2460:
2459:
2449:
2425:
2419:
2418:
2408:
2384:
2375:
2374:
2346:
2340:
2339:
2332:
2326:
2325:
2315:
2298:(4): 1465–1473.
2283:
2277:
2276:
2269:
2263:
2262:
2255:
2249:
2248:
2220:
2214:
2213:
2203:
2194:(8): 1233–1239.
2179:
2173:
2172:
2143:
2132:
2131:
2113:
2102:
2101:
2091:
2081:
2057:
2048:
2047:
2019:
2013:
2012:
1984:
1971:
1970:
1945:(8): 1847–1855.
1934:
1919:
1918:
1916:
1914:
1904:
1895:
1894:
1883:
1877:
1876:
1865:
1859:
1849:
1838:
1828:
1743:Genetic location
1736:Candida glabrata
1600:
1450:
1399:
1247:
1243:
1059:
1058:
1030:
1023:
1006:
996:
987:
959:
955:RefSeq (protein)
945:
929:
920:
888:
866:
847:
821:
802:
776:
757:
726:
516:
495:
480:
456:digastric muscle
420:
418:Top expressed in
413:
358:
356:Top expressed in
351:
330:
313:
303:
290:
279:
264:
257:
251:
240:
228:
212:
202:
189:
178:
163:
156:
150:
139:
125:
109:
103:
54:
47:
33:
21:
4487:
4486:
4482:
4481:
4480:
4478:
4477:
4476:
4447:
4446:
4445:
4433:
4425:
4423:
4418:
4330:Oxidoreductases
4316:
4292:Enzyme kinetics
4280:
4276:List of enzymes
4249:
4218:
4189:Catalytic triad
4167:
4162:
4132:
4127:
4104:
4089:
4045:
4018:
3985:
3960:Pertussis toxin
3909:
3894:
3817:
3739:
3591:
3558:
3484:
3473:
3459:
3429:
3424:
3408:
3395:
3198:
3141:
3087:
3077:
3047:
3042:
3017:
2993:
2972:extralysosomal:
2960:
2924:
2904:
2852:
2847:
2811:
2810:
2806:
2768:
2767:
2763:
2719:
2718:
2709:
2702:
2689:
2688:
2684:
2654:
2653:
2649:
2597:
2596:
2592:
2562:
2561:
2557:
2526:
2525:
2518:
2509:
2508:
2504:
2468:
2467:
2463:
2427:
2426:
2422:
2386:
2385:
2378:
2348:
2347:
2343:
2334:
2333:
2329:
2285:
2284:
2280:
2271:
2270:
2266:
2257:
2256:
2252:
2222:
2221:
2217:
2181:
2180:
2176:
2145:
2144:
2135:
2128:
2115:
2114:
2105:
2059:
2058:
2051:
2021:
2020:
2016:
1986:
1985:
1974:
1936:
1935:
1922:
1912:
1910:
1906:
1905:
1898:
1885:
1884:
1880:
1867:
1866:
1862:
1850:
1841:
1829:
1820:
1816:
1777:gene expression
1765:
1759:
1745:
1711:In mammals and
1709:
1661:
1656:
1160:
1068:View/Edit Mouse
1063:View/Edit Human
1026:
1019:
1016:Location (UCSC)
1004:
983:
979:
975:
971:
967:
941:
937:
916:
910:
906:
902:
898:
894:
811:ENSG00000162688
704:
653:
597:
548:protein binding
476:
467:
462:
458:
454:
450:
446:
444:temporal muscle
442:
438:
434:
430:
428:muscle of thigh
416:
405:
400:
396:
392:
388:
384:
380:
376:
372:
368:
354:
298:
285:
277:
267:
266:
265:
258:
238:
215:Gene location (
197:
184:
176:
166:
165:
164:
157:
135:
112:Gene location (
101:AGL - orthologs
63:
50:
43:
17:
12:
11:
5:
4485:
4483:
4475:
4474:
4469:
4464:
4459:
4449:
4448:
4444:
4443:
4420:
4419:
4417:
4416:
4403:
4390:
4377:
4364:
4351:
4338:
4324:
4322:
4318:
4317:
4315:
4314:
4309:
4304:
4299:
4294:
4288:
4286:
4282:
4281:
4279:
4278:
4273:
4268:
4263:
4257:
4255:
4254:Classification
4251:
4250:
4248:
4247:
4242:
4237:
4232:
4226:
4224:
4220:
4219:
4217:
4216:
4211:
4206:
4201:
4196:
4191:
4186:
4181:
4175:
4173:
4169:
4168:
4163:
4161:
4160:
4153:
4146:
4138:
4129:
4128:
4126:
4125:
4120:
4115:
4109:
4107:
4095:
4094:
4091:
4090:
4088:
4087:
4086:
4085:
4075:
4074:
4073:
4068:
4057:
4055:
4051:
4050:
4047:
4046:
4044:
4043:
4042:
4041:
4026:
4024:
4020:
4019:
4017:
4016:
4011:
4006:
4001:
3995:
3993:
3987:
3986:
3984:
3983:
3977:
3976:
3970:
3969:
3968:
3967:
3962:
3951:
3950:
3949:
3948:
3943:
3932:
3930:
3921:
3912:
3900:
3899:
3896:
3895:
3893:
3892:
3887:
3882:
3877:
3872:
3867:
3862:
3856:
3855:
3850:
3845:
3844:
3843:
3838:
3827:
3825:
3819:
3818:
3816:
3815:
3810:
3805:
3800:
3795:
3790:
3785:
3780:
3775:
3770:
3765:
3760:
3755:
3749:
3747:
3741:
3740:
3738:
3737:
3732:
3727:
3717:
3716:
3711:
3706:
3701:
3696:
3691:
3686:
3681:
3676:
3671:
3665:
3664:
3659:
3654:
3649:
3644:
3639:
3634:
3629:
3624:
3618:
3617:
3612:
3607:
3601:
3599:
3593:
3592:
3590:
3589:
3579:
3574:
3568:
3566:
3560:
3559:
3557:
3556:
3551:
3546:
3541:
3536:
3531:
3526:
3525:
3524:
3519:
3514:
3509:
3498:
3496:
3487:
3475:
3474:
3460:
3458:
3457:
3450:
3443:
3435:
3426:
3425:
3423:
3422:
3412:
3410:
3401:
3400:
3397:
3396:
3394:
3393:
3392:
3391:
3381:
3376:
3371:
3370:
3369:
3364:
3357:Hexosaminidase
3354:
3349:
3344:
3343:
3342:
3332:
3327:
3326:
3325:
3320:
3310:
3305:
3300:
3299:
3298:
3288:
3283:
3282:
3281:
3276:
3269:Galactosidases
3266:
3265:
3264:
3259:
3254:
3249:
3244:
3239:
3229:
3224:
3219:
3218:
3217:
3206:
3204:
3200:
3199:
3197:
3196:
3191:
3190:
3189:
3179:
3178:
3177:
3172:
3167:
3157:
3151:
3149:
3143:
3142:
3140:
3139:
3134:
3129:
3124:
3110:
3108:
3106:Disaccharidase
3099:
3089:
3088:
3078:
3076:
3075:
3068:
3061:
3053:
3044:
3043:
3041:
3040:
3035:
3029:
3027:
3023:
3022:
3019:
3018:
3016:
3015:
3004:
3002:
2995:
2994:
2992:
2991:
2986:
2981:
2975:
2973:
2966:
2965:Glycogenolysis
2962:
2961:
2959:
2958:
2953:
2948:
2943:
2938:
2932:
2930:
2926:
2925:
2919:glycogenolysis
2905:
2903:
2902:
2895:
2888:
2880:
2874:
2873:
2863:
2858:
2851:
2850:External links
2848:
2846:
2845:
2824:(7): 446–463.
2804:
2777:(3): 218–226.
2761:
2732:(2): 352–357.
2707:
2700:
2682:
2647:
2590:
2571:(5): 627–634.
2555:
2516:
2502:
2461:
2420:
2376:
2357:(1–2): 76–85.
2341:
2327:
2278:
2264:
2250:
2231:(6): 885–892.
2215:
2174:
2155:(4): 913–922.
2133:
2126:
2103:
2049:
2030:(3): 257–264.
2014:
1995:(2): 155–165.
1972:
1920:
1896:
1878:
1860:
1839:
1817:
1815:
1812:
1800:cardiomyopathy
1761:Main article:
1758:
1755:
1744:
1741:
1708:
1705:
1660:
1657:
1655:
1652:
1649:
1648:
1645:
1644:
1641:
1640:
1635:
1629:
1628:
1623:
1617:
1616:
1611:
1605:
1604:
1596:
1595:
1586:
1580:
1579:
1568:
1561:
1560:
1555:
1549:
1548:
1543:
1537:
1536:
1531:
1525:
1524:
1519:
1513:
1512:
1507:
1501:
1500:
1495:
1489:
1488:
1484:
1483:
1478:
1472:
1471:
1466:
1460:
1459:
1455:
1454:
1447:
1444:
1443:
1440:
1439:
1434:
1428:
1427:
1422:
1416:
1415:
1410:
1404:
1403:
1395:
1394:
1385:
1379:
1378:
1367:
1360:
1359:
1354:
1348:
1347:
1342:
1336:
1335:
1330:
1324:
1323:
1318:
1312:
1311:
1306:
1300:
1299:
1294:
1288:
1287:
1283:
1282:
1277:
1271:
1270:
1265:
1259:
1258:
1254:
1253:
1238:
1237:
1195:
1162:Together with
1159:
1156:
1103:phosphorylases
1101:Together with
1075:
1074:
1071:
1070:
1065:
1055:
1054:
1048:
1047:
1044:
1043:
1041:
1039:
1032:
1031:
1024:
1017:
1013:
1012:
1009:
1008:
1000:
999:
993:
990:
989:
963:
962:
956:
952:
951:
948:
947:
933:
932:
926:
923:
922:
912:
911:
885:
881:
880:
877:
876:
870:
869:
863:
860:
859:
851:
850:
844:
838:
837:
834:
833:
825:
824:
818:
815:
814:
806:
805:
799:
793:
792:
789:
788:
780:
779:
773:
770:
769:
761:
760:
754:
748:
747:
742:
737:
733:
732:
722:
721:
718:
717:
706:
705:
703:
702:
697:
692:
687:
682:
677:
672:
667:
661:
659:
655:
654:
652:
651:
646:
644:inclusion body
641:
636:
631:
626:
621:
616:
611:
605:
603:
599:
598:
596:
595:
590:
585:
580:
575:
570:
565:
560:
555:
550:
545:
540:
535:
529:
527:
523:
522:
512:
511:
508:
507:
504:
503:
500:
499:
492:
486:
485:
482:
481:
473:
472:
469:
468:
466:
465:
461:
457:
453:
449:
445:
441:
437:
433:
429:
425:
422:
421:
410:
407:
406:
404:
403:
399:
395:
394:deltoid muscle
391:
387:
386:body of tongue
383:
379:
375:
371:
370:biceps brachii
367:
363:
360:
359:
347:
346:
338:
327:
321:
320:
317:RNA expression
309:
308:
305:
304:
296:
292:
291:
283:
280:
275:
269:
268:
259:
252:
246:
242:
241:
236:
230:
229:
221:
220:
208:
207:
204:
203:
195:
191:
190:
182:
179:
174:
168:
167:
158:
151:
145:
141:
140:
133:
127:
126:
118:
117:
105:
104:
61:
57:
56:
48:
40:
39:
35:
34:
26:
25:
15:
13:
10:
9:
6:
4:
3:
2:
4484:
4473:
4470:
4468:
4465:
4463:
4460:
4458:
4455:
4454:
4452:
4442:
4437:
4432:
4428:
4414:
4410:
4409:
4404:
4401:
4397:
4396:
4391:
4388:
4384:
4383:
4378:
4375:
4371:
4370:
4365:
4362:
4358:
4357:
4352:
4349:
4345:
4344:
4339:
4336:
4332:
4331:
4326:
4325:
4323:
4319:
4313:
4310:
4308:
4305:
4303:
4300:
4298:
4295:
4293:
4290:
4289:
4287:
4283:
4277:
4274:
4272:
4271:Enzyme family
4269:
4267:
4264:
4262:
4259:
4258:
4256:
4252:
4246:
4243:
4241:
4238:
4236:
4235:Cooperativity
4233:
4231:
4228:
4227:
4225:
4221:
4215:
4212:
4210:
4207:
4205:
4202:
4200:
4197:
4195:
4194:Oxyanion hole
4192:
4190:
4187:
4185:
4182:
4180:
4177:
4176:
4174:
4170:
4166:
4159:
4154:
4152:
4147:
4145:
4140:
4139:
4136:
4124:
4121:
4119:
4116:
4114:
4111:
4110:
4108:
4106:
4100:
4096:
4084:
4081:
4080:
4079:
4076:
4072:
4069:
4067:
4064:
4063:
4062:
4059:
4058:
4056:
4052:
4040:
4037:
4036:
4035:
4031:
4028:
4027:
4025:
4021:
4015:
4012:
4010:
4007:
4005:
4002:
4000:
3997:
3996:
3994:
3992:
3988:
3982:
3979:
3978:
3975:
3972:
3971:
3966:
3965:Cholera toxin
3963:
3961:
3958:
3957:
3956:
3953:
3952:
3947:
3944:
3942:
3939:
3938:
3937:
3934:
3933:
3931:
3929:
3925:
3922:
3920:
3916:
3913:
3911:
3905:
3901:
3891:
3888:
3886:
3883:
3881:
3878:
3876:
3873:
3871:
3868:
3866:
3863:
3861:
3858:
3857:
3854:
3851:
3849:
3846:
3842:
3839:
3837:
3834:
3833:
3832:
3829:
3828:
3826:
3824:
3820:
3814:
3811:
3809:
3806:
3804:
3801:
3799:
3796:
3794:
3791:
3789:
3786:
3784:
3781:
3779:
3776:
3774:
3771:
3769:
3766:
3764:
3761:
3759:
3756:
3754:
3751:
3750:
3748:
3746:
3742:
3736:
3733:
3731:
3728:
3726:
3722:
3719:
3718:
3715:
3712:
3710:
3707:
3705:
3702:
3700:
3697:
3695:
3692:
3690:
3687:
3685:
3682:
3680:
3677:
3675:
3672:
3670:
3667:
3666:
3663:
3660:
3658:
3655:
3653:
3650:
3648:
3645:
3643:
3640:
3638:
3635:
3633:
3630:
3628:
3625:
3623:
3620:
3619:
3616:
3613:
3611:
3608:
3606:
3603:
3602:
3600:
3598:
3597:Glucuronosyl-
3594:
3587:
3583:
3580:
3578:
3575:
3573:
3570:
3569:
3567:
3565:
3561:
3555:
3552:
3550:
3547:
3545:
3542:
3540:
3537:
3535:
3532:
3530:
3527:
3523:
3520:
3518:
3515:
3513:
3510:
3508:
3505:
3504:
3503:
3502:Phosphorylase
3500:
3499:
3497:
3495:
3491:
3488:
3486:
3480:
3476:
3471:
3467:
3463:
3456:
3451:
3449:
3444:
3442:
3437:
3436:
3433:
3421:
3417:
3414:
3413:
3411:
3407:: Hydrolysing
3406:
3402:
3390:
3387:
3386:
3385:
3382:
3380:
3377:
3375:
3372:
3368:
3365:
3363:
3360:
3359:
3358:
3355:
3353:
3350:
3348:
3345:
3341:
3338:
3337:
3336:
3333:
3331:
3328:
3324:
3323:non-lysosomal
3321:
3319:
3316:
3315:
3314:
3311:
3309:
3306:
3304:
3303:Hyaluronidase
3301:
3297:
3294:
3293:
3292:
3291:Glucuronidase
3289:
3287:
3284:
3280:
3277:
3275:
3272:
3271:
3270:
3267:
3263:
3260:
3258:
3255:
3253:
3250:
3248:
3245:
3243:
3240:
3238:
3235:
3234:
3233:
3232:Neuraminidase
3230:
3228:
3225:
3223:
3220:
3216:
3215:Alpha-amylase
3213:
3212:
3211:
3208:
3207:
3205:
3201:
3195:
3192:
3188:
3185:
3184:
3183:
3180:
3176:
3173:
3171:
3168:
3166:
3163:
3162:
3161:
3158:
3156:
3153:
3152:
3150:
3148:
3144:
3138:
3135:
3133:
3130:
3128:
3125:
3123:
3119:
3115:
3112:
3111:
3109:
3107:
3103:
3100:
3098:
3094:
3090:
3085:
3081:
3074:
3069:
3067:
3062:
3060:
3055:
3054:
3051:
3039:
3036:
3034:
3031:
3030:
3028:
3024:
3013:
3009:
3006:
3005:
3003:
3000:
2996:
2990:
2987:
2985:
2982:
2980:
2977:
2976:
2974:
2970:
2967:
2963:
2957:
2954:
2952:
2949:
2947:
2944:
2942:
2939:
2937:
2934:
2933:
2931:
2927:
2923:
2920:
2916:
2912:
2908:
2901:
2896:
2894:
2889:
2887:
2882:
2881:
2878:
2871:
2867:
2864:
2862:
2859:
2857:
2854:
2853:
2849:
2841:
2837:
2832:
2827:
2823:
2819:
2815:
2808:
2805:
2800:
2796:
2792:
2788:
2784:
2780:
2776:
2772:
2765:
2762:
2757:
2753:
2748:
2743:
2739:
2735:
2731:
2727:
2723:
2716:
2714:
2712:
2708:
2703:
2697:
2693:
2686:
2683:
2678:
2674:
2670:
2666:
2663:(1): 95–100.
2662:
2658:
2651:
2648:
2643:
2639:
2634:
2629:
2625:
2621:
2617:
2613:
2609:
2605:
2601:
2594:
2591:
2586:
2582:
2578:
2574:
2570:
2566:
2559:
2556:
2551:
2547:
2543:
2539:
2535:
2531:
2523:
2521:
2517:
2512:
2506:
2503:
2498:
2494:
2489:
2484:
2480:
2476:
2472:
2465:
2462:
2457:
2453:
2448:
2443:
2439:
2435:
2431:
2424:
2421:
2416:
2412:
2407:
2402:
2398:
2394:
2390:
2383:
2381:
2377:
2372:
2368:
2364:
2360:
2356:
2352:
2345:
2342:
2337:
2331:
2328:
2323:
2319:
2314:
2309:
2305:
2301:
2297:
2293:
2289:
2282:
2279:
2274:
2268:
2265:
2260:
2254:
2251:
2246:
2242:
2238:
2234:
2230:
2226:
2219:
2216:
2211:
2207:
2202:
2197:
2193:
2189:
2185:
2178:
2175:
2170:
2166:
2162:
2158:
2154:
2150:
2142:
2140:
2138:
2134:
2129:
2123:
2119:
2112:
2110:
2108:
2104:
2099:
2095:
2090:
2085:
2080:
2075:
2071:
2067:
2063:
2056:
2054:
2050:
2045:
2041:
2037:
2033:
2029:
2025:
2018:
2015:
2010:
2006:
2002:
1998:
1994:
1990:
1983:
1981:
1979:
1977:
1973:
1968:
1964:
1960:
1956:
1952:
1948:
1944:
1940:
1933:
1931:
1929:
1927:
1925:
1921:
1909:
1903:
1901:
1897:
1892:
1888:
1882:
1879:
1874:
1870:
1864:
1861:
1857:
1853:
1848:
1846:
1844:
1840:
1836:
1832:
1827:
1825:
1823:
1819:
1813:
1811:
1809:
1805:
1801:
1797:
1793:
1789:
1784:
1782:
1778:
1774:
1769:
1764:
1756:
1754:
1751:
1742:
1740:
1737:
1728:
1724:
1722:
1716:
1714:
1706:
1704:
1702:
1701:
1697:
1692:
1690:
1686:
1682:
1680:
1679:maltotetraose
1676:
1672:
1668:
1667:
1658:
1653:
1639:
1636:
1634:
1630:
1627:
1624:
1622:
1618:
1615:
1612:
1610:
1606:
1601:
1597:
1594:
1590:
1587:
1585:
1584:Gene Ontology
1581:
1578:
1575:
1572:
1569:
1566:
1562:
1559:
1556:
1554:
1550:
1547:
1544:
1542:
1538:
1535:
1532:
1530:
1526:
1523:
1522:NiceZyme view
1520:
1518:
1514:
1511:
1508:
1506:
1502:
1499:
1496:
1494:
1490:
1485:
1482:
1479:
1477:
1473:
1470:
1467:
1465:
1461:
1456:
1451:
1448:
1438:
1435:
1433:
1429:
1426:
1423:
1421:
1417:
1414:
1411:
1409:
1405:
1400:
1396:
1393:
1389:
1386:
1384:
1383:Gene Ontology
1380:
1377:
1374:
1371:
1368:
1365:
1361:
1358:
1355:
1353:
1349:
1346:
1343:
1341:
1337:
1334:
1331:
1329:
1325:
1322:
1321:NiceZyme view
1319:
1317:
1313:
1310:
1307:
1305:
1301:
1298:
1295:
1293:
1289:
1284:
1281:
1278:
1276:
1272:
1269:
1266:
1264:
1260:
1255:
1252:
1248:
1245:
1244:
1241:
1235:
1231:
1227:
1223:
1219:
1215:
1211:
1208:
1200:
1196:
1193:
1189:
1185:
1182:
1178:
1177:
1176:
1174:
1169:
1165:
1164:phosphorylase
1157:
1155:
1153:
1149:
1145:
1141:
1137:
1136:
1131:
1126:
1124:
1120:
1116:
1112:
1108:
1104:
1099:
1097:
1093:
1089:
1086:
1082:
1069:
1064:
1060:
1056:
1053:
1049:
1042:
1040:
1037:
1033:
1029:
1025:
1022:
1018:
1014:
1007:
1001:
997:
994:
988:
986:
982:
978:
974:
970:
964:
960:
957:
953:
946:
944:
940:
934:
930:
927:
921:
919:
913:
909:
905:
901:
897:
893:
889:
886:
884:RefSeq (mRNA)
882:
875:
871:
867:
864:
858:
857:
852:
848:
845:
843:
839:
832:
831:
826:
822:
819:
813:
812:
807:
803:
800:
798:
794:
787:
786:
781:
777:
774:
768:
767:
762:
758:
755:
753:
749:
746:
743:
741:
738:
734:
731:
727:
723:
716:
712:
707:
701:
698:
696:
693:
691:
688:
686:
683:
681:
678:
676:
673:
671:
668:
666:
663:
662:
660:
657:
656:
650:
647:
645:
642:
640:
637:
635:
632:
630:
627:
625:
622:
620:
617:
615:
612:
610:
607:
606:
604:
601:
600:
594:
591:
589:
586:
584:
581:
579:
576:
574:
571:
569:
566:
564:
561:
559:
556:
554:
551:
549:
546:
544:
541:
539:
536:
534:
531:
530:
528:
525:
524:
521:
520:Gene ontology
517:
513:
501:
496:
493:
491:
487:
479:
474:
463:
459:
455:
451:
447:
443:
439:
435:
431:
427:
426:
423:
419:
414:
411:
401:
397:
393:
389:
385:
381:
377:
373:
369:
365:
364:
361:
357:
352:
349:
348:
345:
343:
339:
337:
336:
332:
331:
328:
326:
322:
318:
314:
310:
302:
297:
293:
289:
284:
274:
270:
263:
256:
250:
243:
235:
231:
227:
222:
218:
213:
209:
201:
196:
192:
188:
183:
173:
169:
162:
155:
149:
142:
138:
132:
128:
124:
119:
115:
110:
106:
102:
98:
94:
90:
86:
82:
78:
74:
70:
66:
58:
53:
46:
41:
36:
32:
27:
22:
19:
4408:Translocases
4405:
4392:
4379:
4366:
4353:
4343:Transferases
4340:
4327:
4184:Binding site
4105:transferases
3910:transferases
3533:
3485:transferases
3462:Transferases
3193:
3147:Glucosidases
2983:
2929:Glycogenesis
2915:glycogenesis
2821:
2817:
2807:
2774:
2770:
2764:
2729:
2725:
2691:
2685:
2660:
2656:
2650:
2607:
2603:
2593:
2568:
2564:
2558:
2533:
2530:Biochemistry
2529:
2505:
2478:
2474:
2464:
2437:
2433:
2423:
2396:
2392:
2354:
2350:
2344:
2330:
2295:
2291:
2281:
2267:
2253:
2228:
2224:
2218:
2191:
2187:
2177:
2152:
2148:
2118:Biochemistry
2117:
2069:
2065:
2027:
2023:
2017:
1992:
1988:
1942:
1938:
1913:February 29,
1911:. Retrieved
1890:
1881:
1872:
1863:
1792:hypoglycemia
1788:hepatomegaly
1785:
1770:
1766:
1746:
1735:
1733:
1721:active sites
1717:
1710:
1698:
1693:
1684:
1683:
1674:
1670:
1664:
1662:
1510:BRENDA entry
1309:BRENDA entry
1239:
1226:oxocarbenium
1161:
1151:
1143:
1139:
1133:
1127:
1125:can result.
1100:
1087:
1080:
1078:
1003:
966:
943:NM_001362367
939:NM_001081326
936:
915:
873:
854:
828:
809:
783:
764:
744:
739:
340:
333:
299:116,601,815
286:116,533,648
60:External IDs
18:
4179:Active site
3564:Galactosyl-
3374:Iduronidase
3308:Pullulanase
1808:hypertrophy
1659:Two enzymes
1498:IntEnz view
1458:Identifiers
1297:IntEnz view
1257:Identifiers
1234:nucleophile
1222:active site
1218:amino acids
1214:glucosidase
278:3|3 G1
198:99,924,023
185:99,850,361
38:Identifiers
4451:Categories
4382:Isomerases
4356:Hydrolases
4223:Regulation
3517:Cellobiose
3384:Heparanase
3352:Fucosidase
3170:Neutral AB
3026:Regulation
2956:Glycogenin
2907:Metabolism
2513:. UniProt.
2338:. UniProt.
2275:. UniProt.
1858:, May 2017
1837:, May 2017
1814:References
1768:branches.
1689:isoamylase
1567:structures
1534:KEGG entry
1481:9012-47-9
1366:structures
1333:KEGG entry
1280:9032-09-1
1230:hydrolysis
675:metabolism
344:(ortholog)
81:HomoloGene
4261:EC number
3908:Pentosyl-
3823:Mannosyl-
3494:Glucosyl-
3318:lysosomal
3222:Chitinase
3187:cytosolic
3175:Neutral C
3155:Cellulase
3132:Trehalase
3122:Invertase
3080:Hydrolase
2999:lysosomal
2610:: 11229.
1804:uric acid
1487:Databases
1286:Databases
1092:breakdown
985:NP_000637
981:NP_000635
977:NP_000634
973:NP_000633
969:NP_000019
918:NM_000646
908:NM_000645
904:NM_000644
900:NM_000643
896:NM_000642
892:NM_000028
730:Orthologs
609:cytoplasm
89:GeneCards
4472:EC 3.2.1
4467:EC 2.4.1
4285:Kinetics
4209:Cofactor
4172:Activity
3745:Fucosyl-
3512:Glycogen
3483:Hexosyl-
3227:Lysozyme
2840:20631546
2799:24896145
2642:27088557
2585:17317688
2415:11375985
2371:83831481
2322:15687211
2169:18395742
2098:18703518
1989:Genomics
1967:28334066
1959:20187119
1939:Proteins
1854:–
1833:–
1796:myopathy
1773:mutation
1638:proteins
1626:articles
1614:articles
1571:RCSB PDB
1469:3.2.1.33
1437:proteins
1425:articles
1413:articles
1370:RCSB PDB
1268:2.4.1.25
1210:3.2.1.33
1192:residues
1184:2.4.1.25
1158:Function
1119:glucagon
1096:glycogen
1052:Wikidata
709:Sources:
4441:Biology
4395:Ligases
4165:Enzymes
4123:ST8SIA4
3981:Sirtuin
3714:UGT2B28
3709:UGT2B17
3704:UGT2B15
3699:UGT2B11
3694:UGT2B10
3662:UGT1A10
3586:C1GALT1
3210:Amylase
3137:Lactase
3127:Maltase
3114:Sucrase
2922:enzymes
2791:8273986
2756:8755644
2677:2295969
2633:4837477
2612:Bibcode
2497:2961257
2488:1684360
2456:6447697
2245:7712292
2210:9301101
2089:2661413
2044:6078537
2009:8954797
1856:Ensembl
1835:Ensembl
1781:isoform
1696:archaea
1685:E. coli
1675:E. coli
1671:E. coli
1666:E. coli
1593:QuickGO
1558:profile
1541:MetaCyc
1476:CAS no.
1392:QuickGO
1357:profile
1340:MetaCyc
1275:CAS no.
1220:in the
1135:E. coli
1115:insulin
1107:glucose
842:UniProt
797:Ensembl
736:Species
715:QuickGO
639:nucleus
614:cytosol
319:pattern
77:1924809
45:Aliases
4427:Portal
4369:Lyases
4103:Sialyl
4099:2.4.99
3919:Ribose
3758:POFUT2
3753:POFUT1
3689:UGT2B7
3684:UGT2B4
3679:UGT2A3
3674:UGT2A2
3669:UGT2A1
3657:UGT1A9
3652:UGT1A8
3647:UGT1A7
3642:UGT1A6
3637:UGT1A5
3632:UGT1A4
3627:UGT1A3
3622:UGT1A1
3615:B3GAT3
3610:B3GAT2
3605:B3GAT1
3507:Starch
3296:Klotho
2872:(MeSH)
2838:
2797:
2789:
2754:
2747:507437
2744:
2698:
2675:
2640:
2630:
2583:
2550:269742
2548:
2495:
2485:
2454:
2413:
2369:
2320:
2313:545640
2310:
2243:
2208:
2167:
2124:
2096:
2086:
2042:
2007:
1965:
1957:
1798:, and
1621:PubMed
1603:Search
1589:AmiGO
1577:PDBsum
1517:ExPASy
1505:BRENDA
1493:IntEnz
1464:EC no.
1420:PubMed
1402:Search
1388:AmiGO
1376:PDBsum
1316:ExPASy
1304:BRENDA
1292:IntEnz
1263:EC no.
1212:), or
1186:), or
1148:enzyme
1130:enzyme
1038:search
1036:PubMed
856:P35573
752:Entrez
490:BioGPS
398:glutes
177:1p21.2
69:610860
4321:Types
4071:XYLT2
4066:XYLT1
4054:Other
4039:ECGF1
4023:Other
3904:2.4.2
3890:ALG12
3841:POMT2
3836:POMT1
3813:FUT11
3808:FUT10
3479:2.4.1
3405:3.2.2
3389:HPSE2
3274:Alpha
3203:Other
3093:3.2.1
2795:S2CID
2367:S2CID
1963:S2CID
1750:exons
1713:yeast
1553:PRIAM
1352:PRIAM
1111:liver
785:77559
745:Mouse
740:Human
711:Amigo
342:Mouse
335:Human
282:Start
217:Mouse
181:Start
114:Human
4413:list
4406:EC7
4400:list
4393:EC6
4387:list
4380:EC5
4374:list
4367:EC4
4361:list
4354:EC3
4348:list
4341:EC2
4335:list
4328:EC1
3885:ALG9
3880:ALG8
3875:ALG6
3870:ALG3
3865:ALG2
3860:ALG1
3853:DPM3
3848:DPM1
3803:FUT9
3798:FUT8
3793:FUT7
3788:FUT6
3783:FUT5
3778:FUT4
3773:FUT3
3768:FUT2
3763:FUT1
3735:HAS3
3730:HAS2
3725:HAS1
3522:Myo-
3472:2.4)
3367:HEXB
3362:HEXA
3340:NAGA
3279:Beta
3252:NEU4
3247:NEU3
3242:NEU2
3237:NEU1
3165:Acid
3086:3.2)
3012:Acid
2917:and
2836:PMID
2787:PMID
2752:PMID
2696:ISBN
2673:PMID
2638:PMID
2581:PMID
2546:PMID
2493:PMID
2452:PMID
2411:PMID
2318:PMID
2241:PMID
2206:PMID
2165:PMID
2122:ISBN
2094:PMID
2040:PMID
2005:PMID
1955:PMID
1915:2012
1633:NCBI
1574:PDBe
1529:KEGG
1432:NCBI
1373:PDBe
1328:KEGG
1117:and
1085:gene
1079:The
325:Bgee
273:Band
234:Chr.
172:Band
131:Chr.
65:OMIM
2826:doi
2779:doi
2775:120
2742:PMC
2734:doi
2665:doi
2661:116
2628:PMC
2620:doi
2573:doi
2569:141
2538:doi
2483:PMC
2442:doi
2438:255
2401:doi
2397:276
2359:doi
2308:PMC
2300:doi
2296:187
2233:doi
2196:doi
2157:doi
2153:378
2084:PMC
2074:doi
2070:283
2032:doi
1997:doi
1947:doi
1663:In
1609:PMC
1565:PDB
1408:PMC
1364:PDB
1094:of
1088:AGL
1005:n/a
874:n/a
766:178
498:n/a
295:End
194:End
97:OMA
93:AGL
85:536
73:MGI
52:AGL
24:AGL
4453::
4101::
4032::
3906::
3723::
3481::
3470:EC
3464::
3418::
3095::
3084:EC
2913:,
2909::
2834:.
2822:12
2820:.
2816:.
2793:.
2785:.
2773:.
2750:.
2740:.
2730:98
2728:.
2724:.
2710:^
2671:.
2659:.
2636:.
2626:.
2618:.
2606:.
2602:.
2579:.
2567:.
2544:.
2534:16
2532:.
2519:^
2491:.
2479:41
2477:.
2473:.
2450:.
2436:.
2432:.
2409:.
2395:.
2391:.
2379:^
2365:.
2355:26
2353:.
2316:.
2306:.
2294:.
2290:.
2239:.
2227:.
2204:.
2192:61
2190:.
2186:.
2163:.
2151:.
2136:^
2106:^
2092:.
2082:.
2068:.
2064:.
2052:^
2038:.
2026:.
2003:.
1993:38
1991:.
1975:^
1961:.
1953:.
1943:78
1941:.
1923:^
1899:^
1889:.
1871:.
1842:^
1821:^
1790:,
1591:/
1390:/
1207:EC
1181:EC
1154:.
713:/
301:bp
288:bp
200:bp
187:bp
95:;
91::
87:;
83::
79:;
75::
71:;
67::
4429::
4415:)
4411:(
4402:)
4398:(
4389:)
4385:(
4376:)
4372:(
4363:)
4359:(
4350:)
4346:(
4337:)
4333:(
4157:e
4150:t
4143:v
3588:)
3584:(
3468:(
3454:e
3447:t
3440:v
3120:/
3116:/
3072:e
3065:t
3058:v
3014:)
3010:(
3001::
2899:e
2892:t
2885:v
2842:.
2828::
2801:.
2781::
2758:.
2736::
2704:.
2679:.
2667::
2644:.
2622::
2614::
2608:7
2587:.
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2552:.
2540::
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2417:.
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2324:.
2302::
2261:.
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2235::
2229:4
2212:.
2198::
2171:.
2159::
2130:.
2100:.
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2034::
2028:2
2011:.
1999::
1969:.
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99::
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