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: Jean-Marc Alberto, Julien Hamelet, Christophe Noll, Sébastien Blaise, Jean-Pierre Bronowicki, Jean-Louis Guéant, Jean-Maurice Delabar, Nathalie Janel
• Journal: Molecular genetics and metabolism (Mol Genet Metab) Vol. 91 Issue 4 Pg. 396-8 (Aug 2007) ISSN: 1096-7192 [Print] United States
• PMID: 17562377 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• MicroRNAs
• Homocysteine
• Cystathionine beta-Synthase

Topics

• Animals
• Cystathionine beta-Synthase (genetics)
• Disease Models, Animal
• Homocysteine (metabolism)
• Homocystinuria (genetics, metabolism)
• Methylation
• Mice
• MicroRNAs (genetics)