贝特类药物治疗脂肪酸氧化障碍的潜力：经典药物的复兴？
Potential of fibrates in the treatment of fatty acid oxidation disorders: revival of classical drugs?
作者信息
Djouadi F, Aubey F, Schlemmer D, Gobin S, Laforet P, Wanders R J A, Strauss A W, Bonnefont J P, Bastin J
机构信息
INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France.
出版信息
J Inherit Metab Dis. 2006 Apr-Jun;29(2-3):341-2. doi: 10.1007/s10545-006-0266-3.
PMID:16763897
Abstract
Exposure to fibrates leads to normalization of fatty acid oxidation (FAO) in fibroblasts from patients with myopathic forms of CPT2 deficiency or VLCAD deficiency. Correction of FAO is related to a drug-induced increase of residual enzyme activity, and this could provide a new treatment strategy for these disorders.
摘要
接触贝特类药物可使患有肌病型CPT2缺乏症或VLCAD缺乏症患者的成纤维细胞中的脂肪酸氧化（FAO）恢复正常。FAO的纠正与药物诱导的残余酶活性增加有关，这可能为这些疾病提供一种新的治疗策略。
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