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Overview
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ACADSB Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 424 amino acids (34-432) and having a molecular mass of 46.4kDa. ACADSB is fused to a 25 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques. 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.
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Overview