Mice deficient in cystathionine beta synthase display altered homocysteine remethylation pathway

被引：14

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 ; 100201 ;

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

相关论文

共 13 条

[1] Hyperhomocysteinemia activates nuclear factor-κB in endothelial cells via oxidative stress [J].

Au-Yeung, KKW ;

Woo, CWH ;

Sung, FL ;

Yip, JCW ;

Siow, YL ;

O, K .

CIRCULATION RESEARCH, 2004, 94 (01) :28-36

[2] Mild neonatal hypoxia exacerbates the effects of vitamin-deficient diet on homocysteine metabolism in rats [J].

Blaise, S ;

Alberto, JM ;

Nédélec, E ;

Ayav, A ;

Pourié, G ;

Bronowicki, JP ;

Guéant, JL ;

Daval, JL .

PEDIATRIC RESEARCH, 2005, 57 (06) :777-782

[3] In the cystathionine β-synthase knockout mouse, elevations in total plasma homocysteine increase tissue S-adenosylhomocysteine, but responses of S-adenosylmethionine and DNA methylation are tissue specific [J].

Choumenkovitch, SF ;

Selhub, J ;

Bagley, PJ ;

Maeda, N ;

Nadeau, MR ;

Smith, DE ;

Choi, SW .

JOURNAL OF NUTRITION, 2002, 132 (08) :2157-2160

[4] METHIONINE METABOLISM IN MAMMALS [J].

FINKELSTEIN, JD .

JOURNAL OF NUTRITIONAL BIOCHEMISTRY, 1990, 1 (05) :228-237

[5] Hyperhomocysteinemia due to cystathionine beta synthase deficiency induces dysregulation of genes involved in hepatic lipid homeostasis in mice [J].

Hamelet, Julien ;

Demuth, Karine ;

Paul, Jean-Louis ;

Delabar, Jean-Maurice ;

Janel, Nathalie .

JOURNAL OF HEPATOLOGY, 2007, 46 (01) :151-159

[6]

JENCKS DA, 1987, J BIOL CHEM, V262, P2485

[7] Regulatory studies of murine methylenetetrahydrofolate reductase reveal two major promoters and NF-κB sensitivity [J].

Pickell, L ;

Tran, P ;

Leclerc, D ;

Hiscott, J ;

Rozen, R .

BIOCHIMICA ET BIOPHYSICA ACTA-GENE STRUCTURE AND EXPRESSION, 2005, 1731 (02) :104-114

[8] Cystathionine β synthase deficiency promotes oxidative stress, fibrosis, and steatosis in mice liver [J].

Robert, K ;

Nehmé, J ;

Bourdon, E ;

Pivert, GR ;

Friguet, B ;

Delcayre, C ;

Delabar, JM ;

Janel, N .

GASTROENTEROLOGY, 2005, 128 (05) :1405-1415

[9] Altered gene expression in liver from a murine model of hyperhomocysteinemia [J].

Robert, K ;

Chassé, JF ;

Santiard-Baron, D ;

Vayssettes, C ;

Chabli, A ;

Aupetit, J ;

Maeda, N ;

Kamoun, P ;

London, J ;

Janel, N .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2003, 278 (34) :31504-31511

[10] Homocysteine metabolism. [J].

Selhub, J .

ANNUAL REVIEW OF NUTRITION, 1999, 19 :217-246