Coenzyme Q10 reverses pathological phenotype and reduces apoptosis in familial CoQ10 deficiency

被引：130

作者：

Di Giovanni, S

Mirabella, M

Spinazzola, A

Crociani, P

Silvestri, G

Broccolini, A

Tonali, P

Di Mauro, S

Servidei, S

机构：

[1] Catholic Univ, Policlin Gemelli, Inst Neurol, I-00168 Rome, Italy

[2] IRCCS, Neurol Unit, San Giovanni Rotondo, Italy

[3] Columbia Univ, Dept Neurol, H Houston Merrit Clin Res Ctr Muscular Dystrophy, New York, NY USA

来源：

NEUROLOGY | 2001年 / 57卷 / 03期

关键词：

D O I：

10.1212/WNL.57.3.515

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Two brothers with myopathic coenzyme Q(10) (CoQ(10)) deficiency responded dramatically to CoQ(10) supplementation. Muscle biopsies before therapy showed ragged-red fibers, lipid storage, and complex I + III and II + III deficiency. Approximately 30% of myofibers had multiple features of apoptosis. After 8 months of treatment, excessive lipid storage resolved, CoQ(10) level normalized, mitochondrial enzymes increased, and proportion of fibers with TUNEL-positive nuclei decreased to 10%. The authors conclude that muscle CoQ(10) deficiency can be corrected by supplementation of CoQ(10) which appears to stimulate mitochondrial proliferation and to prevent apoptosis.

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页码：515 / 518

页数：4

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