ETFA recombinant protein

Recombinant Human ETFA protein

Cat. Num. AAA1562007

• Gene Names: ETFA; EMA; GA2; MADD
• Purity: >90% as determined by SDS-PAGE
• Synonyms: ETFA; Recombinant Human ETFA protein; Electron transfer flavoprotein subunit alpha; mitochondrial; Alpha-ETF; ETFA recombinant protein

• Host: E Coli
• Purity/Purification: >90% as determined by SDS-PAGE
• Form/Format: Lyophilized. Lyophilized from PBS pH 7.4, 0.2% NLS, 5% Trehalose, 5% Mannose, 1mM EDTA.

• Species: Homo sapiens (Human)
• Protein Construction: A DNA sequence encoding the human ETFA (Ser52-Thr295) was fused with His tag
• Reconstitution: Reconstitute in sterile water for a stock solution.
• Preparation and Storage: Store at 2 to 8 degree C for one week. Store at -20 to -80 degree C for twelve months from the date of receipt. Use a manual defrost freezer and avoid repeated freeze thaw cycles.; In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.

Related Product Information for ETFA recombinant protein

Heterodimeric electron transfer flavoprotein that accepts electrons from several mitochondrial dehydrogenases, including acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). Required for normal mitochondrial fatty acid oxidation and normal amino acid metabolism.

Product Categories/Family for ETFA recombinant protein

Protein

NCBI and Uniprot Product Information

• NCBI GI #: 4503607
• NCBI GeneID: 2108
• NCBI Accession #: NP_000117.1
• NCBI GenBank Nucleotide #: NM_000126.3
• UniProt Accession #: P13804; Q53XN3; B4DT43
• 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 Official Symbol: ETFA
• NCBI Official Synonym Symbols: EMA; GA2; MADD
• 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
• Protein Family: Electron transfer flavoprotein
• UniProt Gene Name: ETFA
• UniProt Synonym Gene Names: Alpha-ETF
• UniProt Entry Name: ETFA_HUMAN

NCBI Description

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 Description

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