GBE1 antibody (AA 101-200)

Catalog No. ABIN1713806

Product Details anti-GBE1 Antibody

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

Binding Specificity: AA 101-200

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GBE1 antibody is un-conjugated

Application: Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunocytochemistry (ICC), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

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: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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: 4 °C,-20 °C

Storage Comment: Shipped at 4°C. Store at -20°C for one year. 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 (GBE1 Products)

Synonyms: glgBII antibody, APBD antibody, GBE antibody, GSD4 antibody, 2310045H19Rik antibody, 2810426P10Rik antibody, D16Ertd536e antibody, 1,4-alpha-glucan branching protein GlgB antibody, 1,4-alpha-glucan branching protein antibody, glycogen branching protein antibody, glycogen branching enzyme antibody, 1,4-alpha-glucan-branching protein antibody, glycogen branching enzyme; BE; 1,4-alpha-D-glucan:1,4-alpha-D-glucan 6-glucosyl-transferase antibody, 1,4-alpha-glucan branching enzyme 1 antibody, glucan (1,4-alpha-), branching enzyme 1 antibody, glgB antibody, ECs4277 antibody, SCO7332 antibody, Synpcc7942_1085 antibody, AZC_4116 antibody, Sros_8169 antibody, Despr_0975 antibody, GBE1 antibody, Gbe1 antibody

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