Acyl-CoA dehydrogenase
From Proteopedia
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== Function == | == 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. | + | '''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. |
== Disease == | == Disease == | ||
| - | Impairment of the activity of ACDH causes a variety of diseases associated with lack of fatty acid metabolism. | + | 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. SCAD deficiency is a recessive disorder of fatty acid β-oxidation. |
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| + | == Structural highlights == | ||
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| + | SCAD is a homodimer with a single FAD binding site. MCAD is a homotetramer with 4 FAD binding sites in the subunits interface and 4 binding sites for acyl-CoA substrate within each monomer. | ||
==3D structures of acyl-CoA dehydrogenase== | ==3D structures of acyl-CoA dehydrogenase== | ||
Revision as of 09:33, 25 October 2015
Contents |
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.
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. SCAD deficiency is a recessive disorder of fatty acid β-oxidation.
Structural highlights
SCAD is a homodimer with a single FAD binding site. MCAD is a homotetramer with 4 FAD binding sites in the subunits interface and 4 binding sites for acyl-CoA substrate within each monomer.
3D structures of acyl-CoA dehydrogenase
Updated on 25-October-2015
