Specifications
Species Reactivity
Mouse; Rat; Human
Immunogen
A synthetic peptide of human AGL is used for rabbit immunization.
Target
Alternative Names
AGL; amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase; amylo 1, 6 glucosidase, 4 alpha glucanotransferase; glycogen debranching enzyme; glycogen storage disease type III; glycogen debrancher; amylo-1, 6-glucosidase, 4-alpha-glucanotransferase; GDE
Product Background
Antigen Description
This gene encodes the glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1,6-glucosidase activity. Mutations in this gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing. Alternatively spliced transcripts encoding different isoforms have been described.
Pathway
Glucose metabolism, organism-specific biosystem; Glycogen Metabolism, organism-specific biosystem; Glycogen breakdown (glycogenolysis), organism-specific biosystem; Metabolic pathways, organism-specific biosystem; Metabolism, organism-specific biosystem; Metabolism of carbohydrates, organism-specific biosystem; Starch and sucrose metabolism, organism-specific biosystem.
Citations
Publication ()
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