AKI70843.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
Calculated MW: 80kDa
NCBI Official Full Name
GBE1, partial
NCBI Official Synonym Full Names
glucan (1,4-alpha-), branching enzyme 1
NCBI Protein Information
1,4-alpha-glucan-branching enzyme
UniProt Protein Name
1,4-alpha-glucan-branching enzyme
UniProt Synonym Protein Names
Brancher enzyme; Glycogen-branching enzyme
UniProt Entry Name
GLGB_HUMAN
NCBI Summary for GBE1
The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease). [provided by RefSeq, Jul 2008]
UniProt Comments for GBE1
GBE1: a monomeric enzyme that functions in glycogen symthesis by catalyzing the formation of alpha 1,6- glucosidic linkages.Required for sufficient glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule and, consequently, in reducing the osmotic pressure within cells. It is most highly expressed in liver and muscle. Deficiency can result in glycogen storage disease IV (Andersen's disease).
Protein type: Carbohydrate Metabolism - starch and sucrose; EC 2.4.1.18; Transferase
Chromosomal Location of Human Ortholog: 3p12.3
Cellular Component: cytosol
Molecular Function: 1,4-alpha-glucan branching enzyme activity; cation binding; hydrolase activity, hydrolyzing O-glycosyl compounds
Biological Process: carbohydrate metabolic process; generation of precursor metabolites and energy; glucose metabolic process; glycogen biosynthetic process; glycogen metabolic process
Disease: Glycogen Storage Disease Iv; Polyglucosan Body Disease, Adult Form
Research Articles on GBE1
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Products associated with anti-GBE1 antibody
Pathways associated with anti-GBE1 antibody
Diseases associated with anti-GBE1 antibody
Organs/Tissues associated with anti-GBE1 antibody
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