ACADSB Enzyme

Description:
|Introduction Short/branched chain specific acyl-CoA dehydrogenase (ACADSB) belongs to the acyl-CoA dehydrogenase family of enzymes which 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 while having the highest affinity for (s)-2-methylbutyryl-CoA, isobutyryl-CoA and 2-methylhexanoyl-CoA as substrates. ACADSB may use valproyl-CoA as substrate and have a role in regulating the metabolic flux of valproic acid in the development of toxicity of this agent. ACADSB gene defects cause the short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), which is an autosomal recessive disorder characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. |Reco|E. coli-derived. A single, non-glycosylated polypeptide chain containing 424 amino acids (34-432) and having a molecular mass of 46.4 kDa. ACADSB is fused to a 25 amino acid His-tag at N-terminus. Purity: > 90.0% as determined by SDS-|The ACADSB solution (0.5 mg/ml) contains 20 mM Tris-HCl buffer (pH 8.0), 0.1 M NaCl, 10% glycerol and 1 mM DTT. Shipping: The product is shipped with ice |Use a manual defrost freezer and avoid repeated freeze-thaw cycles. 12 months from date of receipt, -20 to -70°C as supplied.