Mitochondrial disorders in progressive muscular dystrophies
Abstract
The literature review gives data on the role of mitochondrial disorders in the pathogenesis of different progressive muscular dystrophies. It describes changes in Duchenne, limb-girdle, facial scapulohumeral (Landuzi—Degerina) muscular dystrophies. The review is based on both clinical and experimental animal studies. Along with the implication of mitochondria in the pathogenesis of the diseases, it describes muscular dystrophy treatment options compensating for energy disorders and overcoming oxidative stress and mitochondrial dysfunction. Mitochondrial studies in different muscle diseases hand physicians treatment modalities that fail to lead to recovery, but compensate for disorders caused by mutations in the genetic apparatus.
About the Authors
D. A. Kharlamov
Research Clinical Institute of Pediatrics
Russian Federation
Russian Federation
T. I. Baranich
N.I. Pirogov Russian National Research Medical University, Moscow
Russian Federation
Russian Federation
O. S. Groznova
Research Clinical Institute of Pediatrics
Russian Federation
Russian Federation
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Review
For citations:
Kharlamov D.A., Baranich T.I., Groznova O.S. Mitochondrial disorders in progressive muscular dystrophies. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2014;59(1):28-33. (In Russ.)
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