ACADSB sirna
ACADSB siRNA (Rat)
NCBI and Uniprot Product Information
NCBI Description
catalyzes the alpha, beta-dehydrogenation of valproyl-CoA and other acyl-CoA esters in fatty acid metabolism [RGD, Feb 2006]
Uniprot Description
ACADSB: Has greatest activity toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl- CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD); also known as 2-methylbutyryl-CoA dehydrogenase deficiency or 2- methylbutyryl glycinuria. SBCADD is an autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features. Belongs to the acyl-CoA dehydrogenase family.
Protein type: Oxidoreductase; EC 1.3.8.5; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - fatty acid; Mitochondrial
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: short-branched-chain-acyl-CoA dehydrogenase activity; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding
Biological Process: acyl-CoA metabolic process; fatty acid beta-oxidation; fatty acid metabolic process