Recovery of MERRF Fibroblasts and Cybrids Pathophysiology by Coenzyme Q10
نویسندگان
چکیده
منابع مشابه
Pathomechanisms in coenzyme q10-deficient human fibroblasts.
Primary coenzyme Q10 (CoQ10) deficiency is a rare mitochondrial disorder associated with 5 major clinical phenotypes: (1) encephalomyopathy, (2) severe infantile multisystemic disease, (3) cerebellar ataxia, (4) isolated myopathy, and (5) steroid-resistant nephrotic syndrome. Growth retardation, deafness and hearing loss have also been described in CoQ10-deficient patients. This heterogeneity i...
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Dilute ethanol (EtOH) is a widely used agent to remove the corneal epithelium during the modern refractive surgery. The application of EtOH may cause the underlying corneal fibroblasts to undergo apoptosis. This study was designed to investigate the protective effect and potential mechanism of the respiratory chain coenzyme Q(10) (CoQ(10)), an electron transporter of the mitochondrial respirato...
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Coenzyme Q10 is a vitamin-like substance used in the treatment of a variety of disorders primarily related to suboptimal cellular energy metabolism and oxidative injury. Studies supporting the efficacy of coenzyme Q10 appear most promising for neurodegenerative disorders such as Parkinson's disease and certain encephalomyopathies for which coenzyme Q10 has gained orphan drug status. Results in ...
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CoQ10 was discovered by Dr. Frederick L. Crane at the University of Wisconsin in the late 1950s during his research on the biochemistry of the mitochondrial electron transport chain, also known as the respiratory chain. The pure substance isolated from beef heart mitochondria was sent to Dr. Karl Folkers at the pharmaceutical company Merck for identification and elucidation of its structure. It...
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ژورنال
عنوان ژورنال: Neurotherapeutics
سال: 2012
ISSN: 1933-7213,1878-7479
DOI: 10.1007/s13311-012-0103-3