title: Mice deficient in cystathionine beta synthase display altered homocysteine remethylation pathway.
abstract: Cystathionine beta synthase (CBS) deficiency is a metabolic disorder that is biochemically characterized by severe hyperhomocysteinemia. In order to show the effects of CBS deficiency onto the activity of the enzymes involved in the remethylation pathway, we used the well characterized genetic model of severe hyperhomocysteinemia in mice. We showed that CBS deficiency in mice reduced hepatic methionine synthase and betaine-homocysteine methyltransferase activities, whereas 5,10-methylene tetrahydrofolate reductase activity was increased.
authors.affiliation: INSERM U724, Faculte de Médecine, Vandoeuvre-lès-Nancy, France.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Alberto
authors.forename: Julien
authors.initials: J
authors.lastname: Hamelet
authors.forename: Christophe
authors.initials: C
authors.lastname: Noll
authors.forename: Sébastien
authors.initials: S
authors.lastname: Blaise
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Bronowicki
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Guéant
authors.forename: Jean-Maurice
authors.initials: JM
authors.lastname: Delabar
authors.forename: Nathalie
authors.initials: N
authors.lastname: Janel
mesh_terms: D000818:Animals; D003541:Cystathionine beta-Synthase / Q000235:genetics; D004195:Disease Models, Animal; D006710:Homocysteine / Q000378:metabolism*; D006712:Homocystinuria / Q000235:genetics* / Q000378:metabolism*; D008745:Methylation; D051379:Mice; D035683:MicroRNAs / Q000235:genetics
chemical_list: D035683:MicroRNAs; D006710:Homocysteine; D003541:Cystathionine beta-Synthase