title: Processing error of type-2 variant isovaleryl-CoA dehydrogenase precursor is caused by inefficient binding to the mitochondrial import receptor.
authors.affiliation: Department of Genetics, Yale University School of Medicine, New Haven, CT 06510.
authors.forename: M
authors.initials: M
authors.lastname: Nagao
authors.forename: J
authors.initials: J
authors.lastname: Vockley
authors.forename: B
authors.initials: B
authors.lastname: Parimoo
authors.forename: K
authors.initials: K
authors.lastname: Tanaka
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D002474:Cell-Free System / Q000502:physiology; D004247:DNA / Q000235:genetics*; D004792:Enzyme Precursors / Q000235:genetics* / Q000378:metabolism; D015971:Gene Expression Regulation, Enzymologic / Q000502:physiology*; D014644:Genetic Variation / Q000235:genetics*; D066298:In Vitro Techniques; D050769:Isovaleryl-CoA Dehydrogenase; D008661:Metabolism, Inborn Errors / Q000201:enzymology / Q000235:genetics*; D008666:Metalloendopeptidases / Q000302:isolation & purification / Q000378:metabolism; D008930:Mitochondria, Liver / Q000201:enzymology; D008969:Molecular Sequence Data; D010088:Oxidoreductases / Q000235:genetics* / Q000378:metabolism; D044925:Oxidoreductases Acting on CH-CH Group Donors; D014176:Protein Biosynthesis / Q000235:genetics; D051381:Rats; D011956:Receptors, Cell Surface / Q000378:metabolism*; D012016:Reference Values; D014158:Transcription, Genetic / Q000235:genetics; D000096122:Mitochondrial Processing Peptidase
chemical_list: D004247:DNA; D004792:Enzyme Precursors; D050769:Isovaleryl-CoA Dehydrogenase; D008666:Metalloendopeptidases; D010088:Oxidoreductases; D044925:Oxidoreductases Acting on CH-CH Group Donors; D011956:Receptors, Cell Surface; D000096122:Mitochondrial Processing Peptidase