GDE Multifunctional enzyme acting as 1,4-alpha-D-glucan:1,4-alpha-D-glucan 4-alpha-D-glycosyltransferase and amylo-1,6-glucosidase in glycogen degradation. Belongs to the glycogen debranching enzyme family. Liver, kidney and lymphoblastoid cells express predominantly isoform 1; whereas muscle and heart express not only isoform 1, but also muscle-specific isoform mRNAs (isoforms 2, 3 and 4). Isoforms 5 and 6 are present in both liver and muscle. 3 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - starch and sucrose; EC; EC; Hydrolase; Transferase; Ubiquitin conjugating system
Chromosomal Location of Human Ortholog: 1p21.2
Cellular Component:  cytoplasm; cytosol; extracellular region; ficolin-1-rich granule lumen; inclusion body; isoamylase complex; nucleus; sarcoplasmic reticulum; secretory granule lumen
Molecular Function:  4-alpha-glucanotransferase activity; amylo-alpha-1,6-glucosidase activity; beta-maltose 4-alpha-glucanotransferase activity; glycogen debranching enzyme activity; polysaccharide binding; polyubiquitin modification-dependent protein binding; protein binding
Biological Process:  glycogen biosynthetic process; glycogen catabolic process; neutrophil degranulation; response to glucocorticoid; response to nutrient
Disease: Glycogen Storage Disease Iii
Reference #:  P35573 (UniProtKB)
Alt. Names/Synonyms: 4-alpha-glucanotransferase; AGL; amylo-1, 6-glucosidase, 4-alpha-glucanotransferase; Amylo-1,6-glucosidase; amylo-1,6-glucosidase, 4-alpha-glucanotransferase; Amylo-alpha-1,6-glucosidase; Dextrin 6-alpha-D-glucosidase; GDE; Glycogen debrancher; Glycogen debranching enzyme; Oligo-1,4-1,4-glucantransferase
Gene Symbols: AGL
Molecular weight: 174,764 Da
Basal Isoelectric point: 6.31  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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