ACADSB: Human short/ branched chain acyl CoA dehydrogenase
PDB Code: 2JIF
The enzyme short/ branched chain acyl CoA dehydrogenase (ACADSB, ACAD7) catalyzes the α/β dehydrogenation of short/ branched chain acyl CoAs like 2-methylbutyryl-CoA derived from isoleucine degradation to the corresponding enoyl-CoA ester (tiglyl-CoA). This constitutes the first step of a β-oxidation pathway leading to the final products propionyl-CoA and acetyl-CoA.
ACADSB is localized to mitochondria, and a homotetrameric enzyme belonging to a FAD dependent acyl-CoA dehydrogenase family. Electron acceptor is the electron transferring protein. ACADSB has greatest activity towards short/ branched chain acyl CoAs, but in addition can metabolize straight chain acyl CoA esters like butyryl-CoA or hexanoyl CoAs. It might also be involved in controlling the metabolic flux of the anticonvulsive drug valproic acid, thus contributing to the toxicity profile of this compound including liver damage (microvesicular steatosis) and induction of oxidative stress.
Defects in ACADSB are the cause of a poorly defined short/ branched-chain acyl-CoA dehydrogenase deficiency (also called 2-methylbutyryl-CoA dehydrogenase deficiency or 2-methylbutyryl glycinuria). This is an autosomal recessive disorder of L-isoleucine catabolism and 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.

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