serw-MX  [xml]  
 


    
 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 .
D08.811.913 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 .
D09 Carbohydrates .
D09.301 Dietary Carbohydrates .
D09.301.915 Starch .
D09.301.915.194 Amylopectin .
D09.698 Polysaccharides .
D09.698.365 Glucans .
D09.698.365.855 Starch .
D09.698.365.855.194 Amylopectin .
 
 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
 
Amylopectin .
A highly branched glucan in starch. .
0.64
7796
 
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. .
0.57
2183