Mice deficient in cystathionine beta synthase display altered homocysteine remethylation pathway

被引：15

作者：

Alberto, Jean-Marc

Hamelet, Julien

Noll, Christophe

Blaise, Sebastien

Bronowicki, Jean-Pierre

Gueant, Jean-Louis

Delabar, Jean-Maurice

Janel, Nathalie

机构：

[1] Univ Paris Diderot, EA 3508, F-75251 Paris 05, France

[2] INSERM, Fac Med, U724, F-54511 Vandoeuvre Les Nancy, France

[3] Univ Paris 05, INSERM, UMR S 747, Paris, France

来源：

MOLECULAR GENETICS AND METABOLISM | 2007年 / 91卷 / 04期

关键词：

cystathionine beta synthase deficiency; mice; homocysteine; liver; methionine synthase; BHMT; MTHFR; activity; gene expression;

D O I：

10.1016/j.ymgme.2007.05.002

中图分类号：

R5 [内科学];

学科分类号：

1002 [临床医学]; 100201 [内科学];

摘要：

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. (c) 2007 Elsevier Inc. All rights reserved.

引用

收藏

页码：396 / 398

页数：3

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