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

FEBS Letters

Aubourg, Patrick¹  Cartier, Nathalie¹  Rocchiccioli, Francis¹  Guidoux, Sylvie¹ 

[1]Inserm U342, Hôpital Saint-Vincent de Paul, 82 avenue Denfert Rochereau, 75014 Paris, France

关键词: Adrenoleukodystrophy;    Very long chain fatty acid;    Statin;   

DOI  :  10.1016/S0014-5793(00)01852-4

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 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.

【 授权许可】

Unknown