ETFDH (electron-transferring-flavoprotein dehydrogenase), also known as electron transfer flavoprotein-ubiquinone oxidoreductase, MADD or ETFQO, is a 617aa 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.
Applications:Suitable for use in Western Blot.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, 0.09% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of human ETFDH. Species Crossreactivity: mouse and rat
Isotype：IgG
Calc Applications Abbrev：IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Recombinant protein corresponding to human ETFDH.
