title: Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway.
abstract: Valine is one of the three branched-chain amino acids which undergoes oxidation within mitochondria. In this paper, we describe the current state of knowledge with respect to the enzymology of the valine oxidation pathway and the different disorders affecting oxidation.
authors.affiliation: Head Lab Genetic Metabolic Diseases, Room F0-226 Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands. r.j.wanders@amc.uva.nl
authors.forename: Ronald J A
authors.initials: RJ
authors.lastname: Wanders
authors.forename: Marinus
authors.initials: M
authors.lastname: Duran
authors.forename: Ference J
authors.initials: FJ
authors.lastname: Loupatty
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000175:diagnosis* / Q000378:metabolism; D000597:Amino Acids, Branched-Chain / Q000378:metabolism*; D000818:Animals; D004746:Enoyl-CoA Hydratase / Q000378:metabolism; D006801:Humans; D006884:Hydroxybutyrate Dehydrogenase / Q000378:metabolism; D050646:Methylmalonate-Semialdehyde Dehydrogenase (Acylating) / Q000378:metabolism; D008928:Mitochondria / Q000378:metabolism; D008956:Models, Chemical; D044925:Oxidoreductases Acting on CH-CH Group Donors / Q000378:metabolism; D010100:Oxygen / Q000737:chemistry*; D013869:Thiolester Hydrolases / Q000378:metabolism; D014633:Valine / Q000737:chemistry / Q000235:genetics*
chemical_list: D000597:Amino Acids, Branched-Chain; D006884:Hydroxybutyrate Dehydrogenase; D050646:Methylmalonate-Semialdehyde Dehydrogenase (Acylating); D044925:Oxidoreductases Acting on CH-CH Group Donors; C038808:2-methylacyl-CoA dehydrogenase; D013869:Thiolester Hydrolases; C035908:3-hydroxyisobutyryl-CoA hydrolase; D004746:Enoyl-CoA Hydratase; D014633:Valine; D010100:Oxygen