Name: Anti-ETFA Antibody (CAB19937)[KO Validated]
Brand: KO Validated Antibodies 2
SKU: CAB19937
Availability: OutOfStock

Antibody Name:	Anti-ETFA Antibody [KO Validated]
Antibody SKU:	CAB19937
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant protein of human ETFA.

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	293T

Immunogen:	Recombinant protein of human ETFA.
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	Email for sequence
Gene ID:	2108
Uniprot:	P13804
Cellular Location:	Mitochondrion matrix
Calculated MW:	30kDa/35kDa
Observed MW:	30KDa

Anti-ETFA Antibody [KO Validated] (CAB19937)

Western blot analysis of extracts from normal (control) and ETFA knockout (KO) 293T cells, using ETFA antibody (CAB19937) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.

Synonyms:

ETFA, EMA, GA2, MADD

Background:

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.

UniProt Protein Function:	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.
UniProt Protein Details:	Protein type:Mitochondrial Chromosomal Location of Human Ortholog: 15q23-q25 Cellular Component: mitochondrial matrix; mitochondrion Molecular Function:acyl-CoA binding; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding; oxidoreductase activity Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis Disease: Multiple Acyl-coa Dehydrogenase Deficiency
NCBI Summary:	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 Code:	P13804
NCBI GenInfo Identifier:	119636
NCBI Gene ID:	2108
NCBI Accession:	P13804.1
UniProt Secondary Accession:	P13804,Q53XN3, B4DT43,
UniProt Related Accession:	P13804
Molecular Weight:	30,026 Da
NCBI Full Name:	Electron transfer flavoprotein subunit alpha, mitochondrial
NCBI Synonym Full Names:	electron transfer flavoprotein alpha subunit
NCBI Official Symbol:	ETFA
NCBI Official Synonym Symbols:	EMA; GA2; MADD
NCBI Protein Information:	electron transfer flavoprotein subunit alpha, mitochondrial
UniProt Protein Name:	Electron transfer flavoprotein subunit alpha, mitochondrial
Protein Family:	Electron transfer flavoprotein
UniProt Gene Name:	ETFA
UniProt Entry Name:	ETFA_HUMAN