ETFB recombinant protein
Recombinant Human ETFB protein, N-His Tag
In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes electron-transfer-flavoprotein, beta polypeptide, which shuttles electrons between primary flavoprotein dehydrogenases involved in mitochondrial fatty acid and amino acid catabolism and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. The gene deficiencies have been implicated in type II glutaricaciduria. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008]
Uniprot Description
ETFB: The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases, including five acyl- CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). Defects in ETFB are the cause of glutaric aciduria type 2B (GA2B). GA2B is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Belongs to the ETF beta-subunit/FixA family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Oxidoreductase; Mitochondrial
Chromosomal Location of Human Ortholog: 19q13.3
Cellular Component: intracellular membrane-bound organelle; mitochondrion; mitochondrial matrix
Molecular Function: electron carrier activity
Biological Process: cellular metabolic process
Disease: Multiple Acyl-coa Dehydrogenase Deficiency