Simvastatin does not normalize very long chain fatty acids in adrenoleukodystrophy mice

被引：19

作者：

Cartier, N ^{[1
]}

Guidoux, S ^{[1
]}

Rocchiccioli, F ^{[1
]}

Aubourg, P ^{[1
]}

机构：

[1] Hop St Vincent de Paul, INSERM U342, F-75014 Paris, France

来源：

FEBS LETTERS | 2000年 / 478卷 / 03期

关键词：

adrenoleukodystrophy; very long chain fatty acid; statin;

D O I：

10.1016/S0014-5793(00)01852-4

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

X-linked adrenoleukodystrophy (ALD) is a genetic demyelinating disorder characterized by accumulation of very long chain fatty acid (VLCFA) in tissues. Lovastatin, an inhibitor of 3-hydroxy-3-methylglutaryl coenzyme A reductase, normalizes VLCFA in fibroblasts and plasma from ALD patients. We dietary treated ALD mice with simvastatin, an analog of lovastatin with similar pharmacokinetics and effects on plasma VLCFA in ALD patients at 20 or 60 mg/kg/day for 6-12 weeks. No decrease of VLCFA content was observed in mouse tissues, including the brain, A significant increase of VLCFA was rather observed in the brain of ALD mice at 60 mg/kg/day, (C) 2000 Federation of European Biochemical Societies. Published by Elsevier Science B.V. All rights reserved.

引用

页码：205 / 208

页数：4

共 32 条

[1]

[Anonymous], METODS ENZYMATIC ANA

[2]

Blum C. B., 1994, AM J CARDIOL, V73, P3

[3]

BRADFORD MM, 1976, ANAL BIOCHEM, V72, P248, DOI 10.1016/0003-2697(76)90527-3

[4] Differential effects of lovastatin on the trafficking of endogenous and lipoprotein-derived cholesterol in human monocyte-derived macrophages [J].

Cignarella, A ;

Brennhausen, B ;

von Eckardstein, A ;

Assmann, G ;

Cullen, P .

ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY, 1998, 18 (08) :1322-1329

[5] Clinical pharmacokinetics of 3-hydroxy-3-methylglutaryl-coenzyme A reductase inhibitors [J].

Desager, JP ;

Horsmans, Y .

CLINICAL PHARMACOKINETICS, 1996, 31 (05) :348-371

[6] Retroviral transfer and long-term expression of the adrenoleukodystrophy gene in human CD34+ cells [J].

Doerflinger, N ;

Miclea, JM ;

Lopez, J ;

Chomienne, C ;

Bougnères, P ;

Aubourg, P ;

Cartier, N .

HUMAN GENE THERAPY, 1998, 9 (07) :1025-1036

[7] The neurobiology of X-linked adrenoleukodystrophy, a demyelinating peroxisomal disorder [J].

Dubois-Dalcq, M ;

Feigenbaum, V ;

Aubourg, P .

TRENDS IN NEUROSCIENCES, 1999, 22 (01) :4-12

[8]

ForssPetter S, 1997, J NEUROSCI RES, V50, P829, DOI 10.1002/(SICI)1097-4547(19971201)50:5<829::AID-JNR19>3.0.CO

[9]

2-W

[10] MUTATIONS IN THE 70K PEROXISOMAL MEMBRANE-PROTEIN GENE IN ZELLWEGER SYNDROME [J].

GARTNER, J ;

MOSER, H ;

VALLE, D .

NATURE GENETICS, 1992, 1 (01) :16-23

← 1 2 3 4 →