Coenzyme Q10 reverses pathological phenotype and reduces apoptosis in familial CoQ10 deficiency.
Neurology
; 57(3): 515-8, 2001 Aug 14.
Article
em En
| MEDLINE
| ID: mdl-11502923
ABSTRACT
Two brothers with myopathic coenzyme Q10 (CoQ10) deficiency responded dramatically to CoQ10 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, CoQ10 level normalized, mitochondrial enzymes increased, and proportion of fibers with TUNEL-positive nuclei decreased to 10%. The authors conclude that muscle CoQ10 deficiency can be corrected by supplementation of CoQ10, which appears to stimulate mitochondrial proliferation and to prevent apoptosis.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Ubiquinona
/
Músculos
/
Doenças Musculares
Limite:
Adolescent
/
Child
/
Child, preschool
/
Humans
/
Male
Idioma:
En
Revista:
Neurology
Ano de publicação:
2001
Tipo de documento:
Article
País de afiliação:
Itália