Gene: [01p21/AGL] 4-alpha-glucanotransferase (EC:2.4.1.25); amylo-1,6-glucosidase (EC:3.2.1.33); glycogen debranching enzyme system (EC:2.4.1.25 & EC:3.2.1.33); glycogen storage disease III (debrancher deficiency; Cori & Forbes diseases);
FUN | In mammals (and yeast), the glycogen debranching enzyme system is expressed by two distinctive but linked enzymatic activities: 1) EC:3.2.1.33 Systematic name: Dextrin 6-alpha-D-glucanohydrolase. Reaction catalyzed: Endohydrolysis of 1,6-alpha-D-glucoside linkages at points of branching in chains of 1,4-linked- -alpha-D-glucose residues. 2) EC:2.4.1.25 Systematic name: 4-alpha-Glucanotransferase Reaction catalyzed: Transfers a segment of a 1,4-alpha-D-glucan to a new position in an acceptor, which may be glucose or a 1,4-alpha-D-glucan." |
REF | IDN,FUN,PEP "Brown DH,
Brown BI: Methods Enzymol, 8, 515-?, 1966 IDN,FUN,PEP "Hehre EJ: Adv Enzymol RSB, 11, 297-?, 1951 FUN,PEP "Lee EYC &: Biochemistry, 9, N11, 2347-2355, 1970 IDN,FUN,PEP "Lukomskaya IS: Dokl Akad Nauk SSSR, 129, 1172-?, 1959 FUN,PEP "Pazur JH, Okada S: JBC, 243, N18, 4732-4738, 1968 SEQ "Yang B-Z &: JBC, 267, 9294-9299, 1992 LOC,MOL "Yang-Feng TL &: Genomics, 13, N4 (Aug), 931-934, 1992 |
SWI | SWISSPROT: P35573 |
KEY | carb, mtbd, lys |
CLA | coding, basic |
LOC | 01 p21 |
MIM | MIM: 232400 |
EZN | ENZYME: 2.4.1.25 |
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