GBE1
GBE1 (1,4-alpha-glucan branching enzyme 1) is the glycogen-branching enzyme that builds alpha-1,6-glucosidic branches into glycogen. It acts alongside glycogenin and glycogen synthase in glycogen biosynthesis, and mutations in the GBE1 gene are linked to glycogen storage disease IV. Browse Assay Genie's current range of GBE1 research reagents below, or read on for the biology behind the target.
GBE1 is a glycogen-branching enzyme that participates in the glycogen biosynthetic process together with glycogenin and glycogen synthase. It catalyses 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 neighbouring chain. This generates alpha-1,6-glucosidic branches from alpha-1,4-linked glucose chains.
Branching matters because it increases the solubility of the glycogen polymer. In doing so it reduces the osmotic pressure within cells, which is why the reaction is considered essential for storing glucose as glycogen. The GBE1 protein belongs to the glycosyl hydrolase 13 family, GlgB subfamily, and is assigned EC 2.4.1.18. According to NCBI Gene, the highest levels of this enzyme are found in liver and muscle.
The human GBE1 gene was previously named glucan (1,4-alpha-), branching enzyme 1. Mutations in the gene are associated with glycogen storage disease IV, also known as Andersen's disease, which makes GBE1 a central subject in studies of inherited disorders of glycogen metabolism. A mouse ortholog, Gbe1, is recorded, and researchers working with a GBE1 antibody or GBE1 ELISA kit should consider which species and sample context suit their question.
GBE1 works with glycogenin and glycogen synthase to build glycogen, making it a key enzyme for studying how branched glycogen polymers are made.
Mutations in GBE1 are associated with glycogen storage disease IV (Andersen's disease), so the enzyme is central to work on this disorder.
The highest levels of this enzyme are found in liver and muscle, tissues where glycogen storage is central to energy handling.
Branching increases glycogen solubility and reduces osmotic pressure within cells, linking GBE1 activity to the physical properties of stored glucose.
GBE1 is a glycogen-branching enzyme. It transfers alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position, creating branches. This increases glycogen solubility and reduces osmotic pressure within cells. It acts with glycogenin and glycogen synthase in glycogen biosynthesis.
The approved name is 1,4-alpha-glucan branching enzyme 1, and UniProt lists it as 1,4-alpha-glucan-branching enzyme. The previous HGNC name was glucan (1,4-alpha-), branching enzyme 1. It is also described simply as glycogen-branching enzyme, and no alternative gene symbols are recorded.
Yes. Mutations in the GBE1 gene are associated with glycogen storage disease IV, also known as Andersen's disease. A mouse ortholog, Gbe1, is recorded, so check the datasheet of any product for its validated reactivity.
Other glycogen metabolism enzymes and related targets with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt Q04446; product specifications from Assay Genie datasheets.