Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial (ACADSB)

Has greatest action toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA in addition to toward short straight chain acyl-CoAs like 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 the agent.

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