perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”perinatology-29Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEWSМитохондриальные нарушения при прогрессирующих мышечных дистрофияхMitochondrial disorders in progressive muscular dystrophiesХарламовД. А.KharlamovD. A.

к.м.н., в.н.с. отделения психоневрологии и эпилептологии; руководитель Центра нервно-мышечной патологии

БараничТ. И.BaranichT. I.

асп. каф. гистологии, эмбриологии и цитологии

ГрозноваО. С.GroznovaO. S.

к.м.н., в.н.с. отделения патологии сердечнососудистой системы

Научно-исследовательский клинический институт педиатрииРоссияResearch Clinical Institute of PediatricsRussian FederationРоссийский национальный исследовательский медицинский университет им. Н.И. Пирогова, МоскваРоссияN.I. Pirogov Russian National Research Medical University, MoscowRussian Federation2014020320165912833Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20162016Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/29

В обзоре литературы приводятся данные о роли митохондриальных нарушений в патогенезе различных прогрессирующих мышечных дистрофий. Описываются изменения при миодистрофиях Дюшенна, конечностно-поясных, лицелопаточно-плечевой (Ландузи—Дежерина). Обзор основан как на клинических, так и на экспериментальных исследованиях, проведенных на моделях животных. Наряду с участием митохондрий в патогенезе заболеваний описываются пути лечения миодистрофий, основанные на компенсации энергетических нарушений, преодолении окислительного стресса и нарушений функции митохондрий. Изучение митохондрий при различных заболеваниях мышц дает в руки врачей методы лечения, не приводящие к излечению, но позволяющие компенсировать нарушения, вызванные мутациями генетического аппарата.

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. 

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The authors declare that there are no conflicts of interest present.