NP_000117.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
35,080 Da
NCBI Official Full Name
electron transfer flavoprotein subunit alpha, mitochondrial isoform a
NCBI Official Synonym Full Names
electron-transfer-flavoprotein, alpha polypeptide
NCBI Protein Information
electron transfer flavoprotein subunit alpha, mitochondrial; alpha-ETF; glutaric aciduria II; electron transfer flavoprotein alpha-subunit; electron transfer flavoprotein, alpha polypeptide
UniProt Protein Name
Electron transfer flavoprotein subunit alpha, mitochondrial
UniProt Synonym Gene Names
UniProt Entry Name
ETFA_HUMAN
NCBI Summary for ETFalpha
ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
UniProt Comments for ETFalpha
ETFA: 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 ETFA are the cause of glutaric aciduria type 2A (GA2A); also known as glutaricaciduria IIA. GA2A 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 alpha-subunit/FixB family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Mitochondrial
Chromosomal Location of Human Ortholog: 15q23-q25
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: FAD binding; electron carrier activity; oxidoreductase activity
Biological Process: cellular metabolic process
Disease: Multiple Acyl-coa Dehydrogenase Deficiency
Research Articles on ETFalpha
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Products associated with ETFalpha elisa kit
Pathways associated with ETFalpha elisa kit
Diseases associated with ETFalpha elisa kit
Organs/Tissues associated with ETFalpha elisa kit
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