Anti-ETFA Antibody Picoband® fluoro594 Vervoeging

The human ETFA gene encodes the Electron-transfer-flavoprotein, alpha subunit, also known as ETF-α. Together with Electron-transfer-flavoprotein, beta subunit, encoded by the 'ETFB' gene, it forms the heterodimericElectron transfer flavoprotein (ETF) . 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.

Phosphopantothenate--cysteine ligase, Phosphopantothenoylcysteine synthetase, PPC synthetase, PPCS, COAB

ETFA

2108

P13804

Human, Mouse, Rat

No cross-reactivity with other proteins.

E.coli-derived human ETFA recombinant protein (Position: M1-K333) .

Expressed in heart, placenta, skeletal muscle kidney, pancreas, spleen, prostate, testis, ovary, ileum and colon. Expressed in lung endothelial and smooth muscle cells (at protein level) .

Flow Cytometry

Amino Acid Metabolism, Amino Acids, Cofactors, Vitamins/Minerals, Metabolic Signaling Pathways, Metabolism, Pathways and Processes, Signal Transduction, Vitamins/Minerals

Immunogen affinity purified.

Liquid

Catalyzes the first step in the biosynthesis of coenzyme A from vitamin B5, where cysteine is conjugated to 4'-phosphopantothenate to form 4-phosphopantothenoylcysteine.

1. Barton, D. E., Yang-Feng, T. L., Finocchiaro, G., Ozasa, H., Tanaka, K., Francke, U. Short chain acyl-CoA dehydrogenase (ACADS) maps to chromosome 12 (q22-qter) and electron transfer flavoprotein (ETFA) to 15 (q23-q25) . (Abstract) Cytogenet. Cell Genet. 46: 577-578, 1987. 2. Finocchiaro, G., Ikeda, Y., Barton, D., Francke, U., Tanaka, K. Molecular cloning and gene mapping of alpha-subunit of human electron transfer flavoprotein. (Abstract) Am. J. Hum. Genet. 41: A214, 1987. 3. Finocchiaro, G., Ito, M., Ikeda, Y., Tanaka, K. Molecular cloning and nucleotide sequence of cDNAs encoding the alpha-subunit of human electron transfer flavoprotein. J. Biol. Chem. 263: 15773-15780, 1988.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

48608 MW

6

Phosphopantothenoylcysteine synthetase

Cytosol.

Serine protease HTRA3

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.