GBE1 Antikörper (AA 101-200) (Biotin)

Produktnummer ABIN1700304

Produktdetails anti-GBE1 Antikörper (Biotin)

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

Bindungsspezifität: AA 101-200

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GBE1 Antikörper ist konjugiert mit Biotin

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

Homologie: Human,Mouse,Rat,Rabbit

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GBE1

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C for 12 months.

Haltbarkeit: 12 months

Details zu GBE1

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

Andere Bezeichnung: GBE1 (GBE1 Produkte)

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

Hintergrund:

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.

Gen-ID: 2632

Pathways: Cellular Glucan Metabolic Process