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Impaired Muscle Mitochondrial Biogenesis and Myogenesis in Spinal Muscular Atrophy

2015; American Medical Association; Volume: 72; Issue: 6 Linguagem: Inglês

10.1001/jamaneurol.2015.0178

2168-6157

Michela Ripolone, Dario Ronchi, Raffaella Violano, Dionis Vallejo, Gigliola Fagiolari, Emanuele Barca, Valeria Lucchini, Irene Colombo, Luísa Villa, Angela Berardinelli, Umberto Balottin, Lucia Morandi, Marina Mora, Andreina Bordoni, Francesco Fortunato, Stefania Corti, Daniela Parisi, António Toscano, Monica Sciacco, Salvatore DiMauro, Giacomo P. Comi, Maurizio Moggio,

Muscle Physiology and Disorders

The important depletion of mitochondrial DNA (mtDNA) and the general depression of mitochondrial respiratory chain complex levels (including complex II) have been confirmed, implying an increasing paucity of mitochondria in the muscle from patients with types I, II, and III spinal muscular atrophy (SMA-I, -II, and -III, respectively).