title: Isolation and expression of a cDNA encoding the precursor for a novel member (ACADSB) of the acyl-CoA dehydrogenase gene family.
abstract: The acyl-CoA dehydrogenases (ACDs) are a family of mitochondrial enzymes that oxidize straight chain or branched chain acyl-CoAs in the metabolism of fatty acids or branched chain amino acids. Deficiencies in members of this gene family are important causes of human disease. A cDNA encoding the human precursor for a novel member (gene symbol ACADSB) of the ACD gene family has been isolated and characterized. The open reading frame of 1.3 kb encodes a precursor protein of 431 amino acids, which is processed in vitro to yield a mature protein of 399 amino acids. The cDNA has significant sequence similarity to other members of the acyl-CoA dehydrogenase family, with the greatest homology (38%) to the short chain acyl-CoA dehydrogenase. The cDNA was expressed in eukaryotic (COS) and prokaryotic (Escherichia coli) cells, producing a protein of the expected size, with activity toward the short branched chain acyl-CoA derivatives ((S)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA), as well as toward the short straight chain acyl-CoAs (butyryl-CoA and hexanoyl-CoA).
authors.affiliation: McGill University-Montreal Children's Hospital Research Institute, Quebec, Canada.
authors.forename: R
authors.initials: R
authors.lastname: Rozen
authors.forename: J
authors.initials: J
authors.lastname: Vockley
authors.forename: L
authors.initials: L
authors.lastname: Zhou
authors.forename: R
authors.initials: R
authors.lastname: Milos
authors.forename: J
authors.initials: J
authors.lastname: Willard
authors.forename: K
authors.initials: K
authors.lastname: Fu
authors.forename: C
authors.initials: C
authors.lastname: Vicanek
authors.forename: L
authors.initials: L
authors.lastname: Low-Nang
authors.forename: E
authors.initials: E
authors.lastname: Torban
authors.forename: B
authors.initials: B
authors.lastname: Fournier
mesh_terms: D042964:Acyl-CoA Dehydrogenase; D044944:Acyl-CoA Dehydrogenases / Q000235:genetics* / Q000378:metabolism; D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D002460:Cell Line; D017931:DNA Primers; D018076:DNA, Complementary; D004792:Enzyme Precursors / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D008969:Molecular Sequence Data; D013379:Substrate Specificity
chemical_list: D017931:DNA Primers; D018076:DNA, Complementary; D004792:Enzyme Precursors; D044944:Acyl-CoA Dehydrogenases; D042964:Acyl-CoA Dehydrogenase