GBE1 antibody (AA 101-200) (AbBy Fluor® 594)
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- Target See all GBE1 Antibodies
- GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))
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Binding Specificity
- AA 101-200
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Reactivity
- Human
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Host
- Rabbit
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Clonality
- Polyclonal
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Conjugate
- This GBE1 antibody is conjugated to AbBy Fluor® 594
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Application
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- Predicted Reactivity
- Human,Mouse,Rat,Rabbit
- Purification
- Purified by Protein A.
- Immunogen
- KLH conjugated synthetic peptide derived from human GBE1
- Isotype
- IgG
- Top Product
- Discover our top product GBE1 Primary Antibody
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- Application Notes
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - Restrictions
- For Research Use only
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- 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
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- 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
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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
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