ETFA Antilichaam - middengebied: Biotine (ARP54290_P050-Biotine)

Electron-transfer-flavoprotein, alpha polypeptide

EMA, GA2, MADD

2108

P13804

NP_000117

Human, Mouse, Rat, Cow, Dog, Goat, Guinea Pig, Horse, Rabbit, Yeast, Zebrafish

The immunogen is a synthetic peptide directed towards the middle region of human ETFA

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.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.

SUMO2; MDM2; PSEN1; PPP6R3; BAG3; TPM1; UBC; SORD; HADHA; ETFB; CDC42; HDAC5; MYC; ICT1; UBA5; DDA1; ATG101

Biotin

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 100%; Dog: 100%; Goat: 86%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%; Yeast: 92%; Zebrafish: 93%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

35kDa

Chiong, M.A., Mol. Genet. Metab. 92 (1-2), 109-114 (2007)

Wet Ice

333

ETFA

ETFA

Rabbit

Electron transfer flavoprotein subunit alpha, mitochondrial

NM_000126

VVSGGRGLKSGENFKLLYDLADQLHAAVGASRAAVDAGFVPNDMQVGQTG

Alpha, mitochondrial