Product Description

ETFDH Polyclonal Antibody

Sizes: 50μl, 100μl

Catalogue Numbers: BS8269-50, BS8269-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: ETFDH (electron-transferring-flavoprotein dehydrogenase), also known as electron transfer flavoprotein-ubiquinone oxidoreductase, MADD or ETFQO, is a 617 amino acid membrane-bound electron transfer protein that exists as a monomer, localizes to the mitochondrial inner membrane and belongs to the ETF-QO/fixC family. ETFDH accepts electrons from electron-transfer flavoprotein (ETF) in the mitochondrial matrix while reducing ubiquinone in the mitochondrial membrane. ETFDH is encoded by a gene mapping to human chromosome 4q32.1, and contains one molecμle of FAD and a 4Fe-4S cluster. As a resμlt of alternative splicing events, two ETFDH isoforms exist. Defects in ETFDH are responsible for an autosomal recessive disorder of amino acid, fatty acid and choline metabolism known as glutaric aciduria type 2C (GA2C) or mμltiple acyl-CoA dehydrogenation deficiency (MADD). GA2C is characterized by severe hypoketotic hypoglycemia and acidosis.

Category: Primary Antibody

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB

Alternate Names: Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial, ETF-QO, ETF-ubiquinone oxidoreductase, Electron-transferring-flavoprotein dehydrogenase, ETF dehydrogenase, ETFDH

Clonality: Polyclonal

Conjugate: Unconjugated

Modification: Unmodification

Immunogen: Recombinant fμll length Human ETFDH.

Dilution: WB 1:500 - 1:2000

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Specificity: ETFDH polyclonal antibody detects endogenous levels of ETFDH protein.

Molecular Weight: ~ 68 kDa

Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

SwissProt: Q16134

Product: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Research Use Only