Biomaterial Database

Structure and mechanism of action of the acyl-CoA dehydrogenases. 1995

C Thorpe, and J J Kim

Department of Chemistry and Biochemistry, University of Delaware, Newark 19716, USA.

Mitochondrial beta-oxidation involves a family of flavoproteins that introduce a C-C double bond into their fatty acyl-CoA substrates. Deficiencies of these acyl-CoA dehydrogenases lead to fatty acid oxidation disorders involving life-threatening episodes of metabolic derangement. This review focuses on the medium chain acyl-CoA dehydrogenase as the best-understood member of its class. The crystal structure of the enzyme and salient features of its substrate specificity and mechanism of action are summarized. The surprising observation of a catalytically essential amino acid residue that nevertheless is not conserved in the acyl-CoA dehydrogenase family is discussed.

UI	MeSH Term	Description	Entries
D008052	Lipid Metabolism, Inborn Errors	Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.	Lipid Metabolism, Inborn Error
D008928	Mitochondria	Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)	Mitochondrial Contraction,Mitochondrion,Contraction, Mitochondrial,Contractions, Mitochondrial,Mitochondrial Contractions
D005420	Flavoproteins		Flavoprotein
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000214	Acyl Coenzyme A	S-Acyl coenzyme A. Fatty acid coenzyme A derivatives that are involved in the biosynthesis and oxidation of fatty acids as well as in ceramide formation.	Acyl CoA,Fatty Acyl CoA,Long-Chain Acyl CoA,Acyl CoA, Fatty,Acyl CoA, Long-Chain,CoA, Acyl,CoA, Fatty Acyl,CoA, Long-Chain Acyl,Coenzyme A, Acyl,Long Chain Acyl CoA
D000818	Animals	Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA.	Animal,Metazoa,Animalia
D001665	Binding Sites	The parts of a macromolecule that directly participate in its specific combination with another molecule.	Combining Site,Binding Site,Combining Sites,Site, Binding,Site, Combining,Sites, Binding,Sites, Combining
D013379	Substrate Specificity	A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.	Specificities, Substrate,Specificity, Substrate,Substrate Specificities
D042964	Acyl-CoA Dehydrogenase	A flavoprotein oxidoreductase that has specificity for medium-chain fatty acids. It forms a complex with ELECTRON TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.	Acyl-coenzyme A Dehydrogenase,Fatty-acyl CoA Dehydrogenase,MCACA-Dehydrogenase,Medium Chain Acyl-CoA Dehydrogenase,Medium-Chain Acyl-CoA Dehydrogenase,Medium-Chain Acyl-Coenzyme A Dehydrogenase,Octanoyl-CoA Dehydrogenase,Palmitoyl-CoA Dehydrogenase,Acyl CoA Dehydrogenase,Acyl coenzyme A Dehydrogenase,Acyl-CoA Dehydrogenase, Medium-Chain,CoA Dehydrogenase, Fatty-acyl,Dehydrogenase, Acyl-CoA,Dehydrogenase, Acyl-coenzyme A,Dehydrogenase, Fatty-acyl CoA,Dehydrogenase, Medium-Chain Acyl-CoA,Dehydrogenase, Octanoyl-CoA,Dehydrogenase, Palmitoyl-CoA,Fatty acyl CoA Dehydrogenase,MCACA Dehydrogenase,Medium Chain Acyl CoA Dehydrogenase,Medium Chain Acyl Coenzyme A Dehydrogenase,Octanoyl CoA Dehydrogenase,Palmitoyl CoA Dehydrogenase
D044944	Acyl-CoA Dehydrogenases	Enzymes that catalyze the first step in the beta-oxidation of FATTY ACIDS.	Acyl CoA Dehydrogenases,Dehydrogenases, Acyl-CoA