Purity: > 90% by SDS - PAGE Format: Liquid. In 20mM Tris-HCl buffer (pH 8.0) containing 0.1M NaCl, 10% glycerol,1mM DTT. Short/branched chain specific acyl-CoA dehydrogenase(ACADSB), is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. ACADSB catalyzes the degradation of L-isoleucine and has the highest affinity for (s)-2-methylbutyryl-CoA, isobutyryl-CoA and 2-methylhexanoyl-CoA as substrates. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), an autosomal recessive disorder characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Recombinant human ACADSB protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.
Size
250ug, 50ug, 10ug
Concentration
n/a
Applications
SDS-PAGE
Other Names
Short/branched chain specific acyl-CoA dehydrogenase mitochondrial, Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial, 2-MEBCAD, ACAD7, SBCAD