Acyl-CoA dehydrogenase
From Proteopedia
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'''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]]. | '''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. | *'''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>. | |
== Disease == | == Disease == | ||
Revision as of 08:42, 21 May 2024
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References
- ↑ Thorpe C, Kim JJ. Structure and mechanism of action of the acyl-CoA dehydrogenases. FASEB J. 1995 Jun;9(9):718-25. PMID:7601336
- ↑ Mohsen AW, Anderson BD, Volchenboum SL, Battaile KP, Tiffany K, Roberts D, Kim JJ, Vockley J. Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia. Biochemistry. 1998 Jul 14;37(28):10325-35. PMID:9665741 doi:10.1021/bi973096r
- ↑ Ibrahim SA, Temtem T. Medium-Chain Acyl-CoA Dehydrogenase Deficiency. PMID:32809672
- ↑ Battaile KP, Molin-Case J, Paschke R, Wang M, Bennett D, Vockley J, Kim JJ. Crystal structure of rat short chain acyl-CoA dehydrogenase complexed with acetoacetyl-CoA: comparison with other acyl-CoA dehydrogenases. J Biol Chem. 2002 Apr 5;277(14):12200-7. Epub 2002 Jan 25. PMID:11812788 doi:10.1074/jbc.M111296200
