GBE1 anticorps (AA 101-200) (Biotin)

N° du produit ABIN1700304

Détail du produit anti-GBE1 anticorps (Biotin)

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

Épitope: AA 101-200

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GBE1 est conjugé à/à la Biotin

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Homologie: Human,Mouse,Rat,Rabbit

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human GBE1

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

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.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C for 12 months.

Date de péremption: 12 months

Détails sur GBE1

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

Autre désignation: GBE1 (GBE1 Produits)

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

Sujet:

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.

ID gène: 2632

Pathways: Cellular Glucan Metabolic Process