Mice deficient in cystathionine beta synthase display altered homocysteine remethylation pathway.

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Date publication

août 2007

Auteurs

Membres identifiés du Cancéropôle Est :
Pr BRONOWICKI Jean-Pierre, Pr GUEANT Jean-Louis


Tous les auteurs :
Alberto JM, Hamelet J, Noll C, Blaise S, Bronowicki JP, Gueant JL, Delabar JM, Janel N

Résumé

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.

Référence

Mol Genet Metab. 2007 Aug;91(4):396-8