Mice deficient in cystathionine beta synthase display altered homocysteine remethylation pathway.
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.
Alberto
Jean-Marc
JM
INSERM U724, Faculte de Médecine, Vandoeuvre-lès-Nancy, France.
Hamelet
Julien
J
Noll
Christophe
C
Blaise
Sébastien
S
Bronowicki
Jean-Pierre
JP
Guéant
Jean-Louis
JL
Delabar
Jean-Maurice
JM
Janel
Nathalie
N
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
D035683:MicroRNAs; D006710:Homocysteine; D003541:Cystathionine beta-Synthase