title: Molecular cloning and characterization of the cDNA encoding the rat liver gamma-butyrobetaine hydroxylase.
abstract: Carnitine biosynthesis from lysine and methionine involves five enzymatic reactions. gamma-butyrobetaine hydroxylase (BBH; EC 1.14. 11.1) is the last enzyme of this pathway. It catalyzes the reaction of hydroxylation of gamma-butyrobetaine to carnitine. The cDNA encoding this enzyme has been isolated and characterized. The cDNA contained an open reading frame of 1161 bp encoding a protein of 387 amino acids with a deduced molecular weight of 44.5 kDa. The sequence of the cDNA showed an important homology with the human cDNA recently isolated. Northern analysis showed gamma-butyrobetaine hydroxylase expression in the liver and in some extend in the testis and the epididymis. During this study, it also appeared that BBH mRNA expression was undetectable by Northern analysis during the perinatal period. During the development of the rat, the amount of BBH mRNA appeared after the weaning of the young rat and reached a maximal expression at the adult stage.
authors.affiliation: Université de Bourgogne, Unité de Recherche en Nutrition Cellulaire et Métabolique, BP47870, F-21078, Dijon, France.
authors.forename: S
authors.initials: S
authors.lastname: Galland
authors.forename: F
authors.initials: F
authors.lastname: Le Borgne
authors.forename: F
authors.initials: F
authors.lastname: Bouchard
authors.forename: B
authors.initials: B
authors.lastname: Georges
authors.forename: P
authors.initials: P
authors.lastname: Clouet
authors.forename: F
authors.initials: F
authors.lastname: Grand-Jean
authors.forename: J
authors.initials: J
authors.lastname: Demarquoy
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D003001:Cloning, Molecular; D018076:DNA, Complementary / Q000737:chemistry / Q000302:isolation & purification; D018507:Gene Expression Regulation, Developmental; D008099:Liver / Q000201:enzymology*; D008297:Male; D006899:Mixed Function Oxygenases / Q000737:chemistry / Q000235:genetics*; D008969:Molecular Sequence Data; D051381:Rats; D017208:Rats, Wistar; D016415:Sequence Alignment; D017386:Sequence Homology, Amino Acid; D012689:Sequence Homology, Nucleic Acid; D050563:gamma-Butyrobetaine Dioxygenase
chemical_list: D018076:DNA, Complementary; D006899:Mixed Function Oxygenases; D050563:gamma-Butyrobetaine Dioxygenase