title: Molecular basis of isovaleric acidemia and the study of the acyl-CoA dehydrogenase family.
authors.affiliation: Department of Human Genetics, Yale University School of Medicine, New Haven, Connecticut 06510.
authors.forename: K
authors.initials: K
authors.lastname: Tanaka
authors.forename: Y
authors.initials: Y
authors.lastname: Ikeda
authors.forename: Y
authors.initials: Y
authors.lastname: Matsubara
authors.forename: D
authors.initials: D
authors.lastname: Hyman
mesh_terms: D044944:Acyl-CoA Dehydrogenases / Q000235:genetics / Q000302:isolation & purification / Q000378:metabolism*; D000818:Animals; D004247:DNA / Q000235:genetics; D045782:Hemiterpenes; D006801:Humans; D007231:Infant, Newborn; D050769:Isovaleryl-CoA Dehydrogenase; D007930:Leucine / Q000378:metabolism; D008930:Mitochondria, Liver / Q000201:enzymology; D010088:Oxidoreductases / Q000172:deficiency* / Q000302:isolation & purification; D044925:Oxidoreductases Acting on CH-CH Group Donors; D010421:Pentanoic Acids / Q000097:blood*; D010641:Phenotype; D014176:Protein Biosynthesis; D011499:Protein Processing, Post-Translational; D051381:Rats; D014631:Valerates / Q000097:blood*
chemical_list: D045782:Hemiterpenes; D010421:Pentanoic Acids; D014631:Valerates; C008216:isovaleric acid; D004247:DNA; D010088:Oxidoreductases; D044944:Acyl-CoA Dehydrogenases; D044925:Oxidoreductases Acting on CH-CH Group Donors; D050769:Isovaleryl-CoA Dehydrogenase; D007930:Leucine