Melanocytes from Patients Affected by Ullrich Congenital Muscular Dystrophy and Bethlem Myopathy have Dysfunctional Mitochondria That Can be Rescued with Cyclophilin Inhibitors

Ullrich congenital muscular dystrophy and Bethlem myopathy are caused by mutations in collagen VI (ColVI) genes, which encode an extracellular matrix protein; yet, mitochondria play a major role in disease pathogenesis through a short circuit caused by inappropriate opening of the permeability trans...

詳細記述

保存先:
書誌詳細
出版年:Front Aging Neurosci
主要な著者: Zulian, Alessandra, Tagliavini, Francesca, Rizzo, Erika, Pellegrini, Camilla, Sardone, Francesca, Zini, Nicoletta, Maraldi, Nadir Mario, Santi, Spartaco, Faldini, Cesare, Merlini, Luciano, Petronilli, Valeria, Bernardi, Paolo, Sabatelli, Patrizia
フォーマット: Artigo
言語:Inglês
出版事項: Frontiers Media S.A. 2014
主題:
Neuroscience
オンライン･アクセス:https://ncbi.nlm.nih.gov/pmc/articles/PMC4238408/
https://ncbi.nlm.nih.gov/pubmed/25477819
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fnagi.2014.00324
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