background:
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.
Function:
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).
Subunit:
Heterodimer of an alpha and a beta subunit.
Subcellular Location:
Mitochondrion matrix.
Tissue Specificity:
Abundant in liver, heart and skeletal muscle. A weak expression is seen in the brain, placenta, lung, kidney and pancreas.
DISEASE:
Defects in ETFB are the cause of glutaric aciduria type 2B (GA2B) [MIM:23336]. 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.
Similarity:
Belongs to the ETF beta-subunit/fixA family.
Database links:
UniProtKB/Swiss-Prot: P38117.3
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
黄素蛋白是由一条多肽结合1个辅基组成的酶类，不是脂溶性。结合的辅基可以是FAD或FMN,它们是维生素B2的衍生物，每个辅基能够接受和提供两个质子和电子。线粒体中的黄素蛋白主要是电子传递链中NADH脱氢酶和TCA循环中的琥珀酸脱氢酶。
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