GBE1 antibody (AA 101-200) (Cy3)

Catalog No. ABIN1702117

This Rabbit Polyclonal antibody specifically detects GBE1 in WB, IF (cc) and IF (p). It exhibits reactivity toward Human.

Quick Overview for GBE1 antibody (AA 101-200) (Cy3) (ABIN1702117)

Target: GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GBE1 antibody is conjugated to Cy3

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Product Details anti-GBE1 Antibody (Cy3)

Binding Specificity: AA 101-200

Predicted Reactivity: Human,Mouse,Rat,Rabbit

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GBE1

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for GBE1

Target: GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))

Alternative Name: GBE1

Background:

Synonyms: 1,4 alpha glucan branching enzyme, 4-alpha-glucan-branching enzyme, amylo 1,4 to 1,6 transglucosidase, amylo 1,4 to 1,6 transglycosylase, Andersen disease, Brancher enzyme, GBE 1, GBE, GBE1, gGlucan 1,4 alpha , branching enzyme 1, GLGB_HUMAN, Glucan 1,4 alpha branching enzyme, Glycogen branching enzyme, Glycogen storage disease type IV, Glycogen-branching enzyme, OTTHUMP00000213788, OTTHUMP00000213833.

Background: GBE1 is a 702 amino acid protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis. Existing as a monomer, GBE1 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 a neighboring glycogen chain and, via this catalytic activity, plays an essential role in glycogen accumulation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood. Unlike GSD4, APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, pyramidal tetraparesis and peripheral neuropathy.

Gene ID: 2632

Pathways: Cellular Glucan Metabolic Process