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 DeCS Categories

C16 Congenital, Hereditary, and Neonatal Diseases and Abnormalities .
C16.320 Genetic Diseases, Inborn .
C16.320.565 Metabolism, Inborn Errors .
C16.320.565.202 Carbohydrate Metabolism, Inborn Errors .
C16.320.565.202.449 Glycogen Storage Disease .
C16.320.565.202.449.540 Glycogen Storage Disease Type IV .
C18 Nutritional and Metabolic Diseases .
C18.452 Metabolic Diseases .
C18.452.648 Metabolism, Inborn Errors .
C18.452.648.202 Carbohydrate Metabolism, Inborn Errors .
C18.452.648.202.449 Glycogen Storage Disease .
C18.452.648.202.449.540 Glycogen Storage Disease Type IV .
D08 Enzymes and Coenzymes .
D08.811 Enzymes .
D08.811.277 Hydrolases .
D08.811.277.450 Glycoside Hydrolases .
D08.811.277.450.420 Glucosidases .
D08.811.277.450.420.450 Glycogen Debranching Enzyme System .
D08.811.913 Transferases .
D08.811.913.400 Glycosyltransferases .
D08.811.913.400.450 Hexosyltransferases .
D08.811.913.400.450.460 Glucosyltransferases .
D08.811.913.400.450.460.100 1,4-alpha-Glucan Branching Enzyme .
D08.811.913.400.450.460.350 Glycogen Debranching Enzyme System .
 
 Terms
 Synonyms & Historicals
Documents
LILACS e MDL
 
1,4-alpha-Glucan Branching Enzyme .
Branching Glycosyltransferase .
Starch Branching Enzyme .
1,4 alpha Glucan Branching Enzyme .
Branching Enzyme, 1,4-alpha-Glucan .
Branching Enzyme, Amylopectin .
Branching Enzyme, Glycogen .
Branching Enzyme, Starch .
Enzyme, 1,4-alpha-Glucan Branching .
Enzyme, Amylopectin Branching .
Enzyme, Branching .
Enzyme, Glycogen Branching .
Enzyme, Starch Branching .
Glycosyltransferase, Branching .
Q Enzyme .
Amylopectin Branching Enzyme .
Glycogen Branching Enzyme .
Q-Enzyme .
Branching Enzyme .
In glycogen or amylopectin synthesis, the enzyme that catalyzes the transfer of a segment of a 1,4-alpha-glucan chain to a primary hydroxy group in a similar glucan chain. EC 2.4.1.18. .
1.00
2427
 
Glycogen Storage Disease Type IV .
Andersen's Disease .
Deficiency, Brancher .
Gbe1 Deficiency .
Glycogen Branching Enzyme Deficiency .
Glycogen Storage Disease Type 4 .
Glycogenosis IV .
Type IV Glycogenosis .
Amylopectinoses .
Andersens Disease .
Brancher Deficiencies .
Deficiencies, Brancher .
Deficiencies, Gbe1 .
Deficiency, Gbe1 .
Disease, Andersen .
Disease, Andersen's .
Gbe1 Deficiencies .
Glycogenoses, Type IV .
Glycogenosis 4s .
Glycogenosis IVs .
Glycogenosis, Type IV .
Type IV Glycogenoses .
Amylopectinosis .
Andersen Disease .
Brancher Deficiency .
Glycogenosis 4 .
An autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal GLYCOGEN with long outer branches. Clinical features are MUSCLE HYPOTONIA and CIRRHOSIS. Death from liver disease usually occurs before age 2. .
0.81
2183
 
Glycogen Debranching Enzyme System .
Debranching Enzyme, Glycogen .
Enzyme, Glycogen Debranching .
Transfer Glucosidase .
Glycogen Debranching Enzyme .
Transfer-Glucosidase .
1,4-alpha-D-Glucan-1,4-alpha-D-glucan 4-alpha-D-glucosyltransferase/dextrin 6 alpha-D-glucanohydrolase. An enzyme system having both 4-alpha-glucanotransferase (EC 2.4.1.25) and amylo-1,6-glucosidase (EC 3.2.1.33) activities. As a transferase it transfers a segment of a 1,4-alpha-D-glucan to a new 4-position in an acceptor, which may be glucose or another 1,4-alpha-D-glucan. As a glucosidase it catalyzes the endohydrolysis of 1,6-alpha-D-glucoside linkages at points of branching in chains of 1,4-linked alpha-D-glucose residues. Amylo-1,6-glucosidase activity is deficient in glycogen storage disease type III. .
0.78
2400