BS8269

antibody from Bioworld Technology, Inc
Targeting: ETFDH ETFQO
Provider product page for BS8269
Western blot
Immunohistochemistry

Antibody data

Product number
BS8269 - Provider product page
Provider
Bioworld Technology, Inc
Product name
ETFDH polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human ETFDH.
Description
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 molecule of FAD and a 4Fe-4S cluster. As a result 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 multiple acyl-CoA dehydrogenation deficiency (MADD). GA2C is characterized by severe hypoketotic hypoglycemia and acidosis.
Reactivity
Human, Mouse, Rat
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
ETFDH protein structure - BS8269 shown in red.