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<StructureSection load='' size='350' side='right' scene='49/491924/Cv/1' caption='Rat short chain acyl-CoA hydrogenase complex with cofactor FAD and CoA (PDB code [[1jqi]])'> __TOC__ == Function == '''Acyl-CoA dehydrogenase''' (ACDH) catalyzes the introduction of a double bond between C2 and C3 of the thio-ester CoA substrate. This is the first reaction in fatty acid metabolism which produces acetyl-CoA. FAD is the cofactor of ACDH activity. ACDH is classified according to the length of its substrates as short- (SCAD), medium- (MCAD), very- and very long-chain (VLCAD) ACDH. MCAD can bind a broad range of chain length acyl-CoA substrates.<ref>PMID:7601336</ref> See also [[Beta oxidation]] and [[Glutaryl-CoA dehydrogenase]]. *'''Glutaryl-CoA dehydrogenase''' is required for the metabolism of Lys, Trp and hydroxyLys. *'''Isovaleryl-CoA dehydrogenase''' catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA<ref>PMID:9665741</ref>. *'''3-hydroxyacyl-CoA dehydrogenase''' oxidates straight chain 3-hydroxyacyl-CoA<ref>PMID:16176262</ref>. == Disease == Impairment of the activity of ACDH causes a variety of diseases associated with lack of fatty acid metabolism. MCAD mutations are associated with Sudden Infant Death and with Medium-chain Acyl-CoA Dehydrogenase Deficiency (MCADD)<ref>PMID:32809672</ref>. For details see [[Investigating the Mechanisms of Active Site Mutations to the 1T9G WT MCAD Protein to Better Understand Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCADD)]]. SCAD deficiency is a recessive disorder of fatty acid β-oxidation. == Structural highlights == SCAD is a homodimer with a single FAD binding site. <scene name='49/491924/Cv/12'>MCAD is a homotetramer</scene> with 4 FAD binding sites in the subunits interface and 4 binding sites for acyl-CoA substrate within each monomer. *One of the <scene name='49/491924/Cv/9'>FAD binding sites</scene> in homotetramer of rat ACDH. Water molecules are shown as red spheres. *One of the <scene name='49/491924/Cv/11'>CoA binding sites</scene> in homotetramer of rat ACDH.<ref>PMID:11812788</ref> ==3D structures of acyl-CoA dehydrogenase== [[Acyl-CoA dehydrogenase 3D structures]] </StructureSection> == References == <references/> [[Category:Topic Page]]

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