Article
Mutant desmin substantially perturbs mitochondrial morphology, function and maintenance in skeletal muscle tissue.
Acta neuropathologica - 1 Sept 2016
Winter Lilli, Wittig Ilka, Peeva Viktoriya, Eggers Britta, Heidler Juliana, Chevessier Frederic, Kley Rudolf A, Barkovits Katalin, Strecker Valentina, Berwanger Carolin, Herrmann Harald, Marcus Katrin, Kornblum Cornelia, Kunz Wolfram S, Schröder Rolf, Clemen Christoph S
Abstract excerpt
Secondary mitochondrial dysfunction is a feature in a wide variety of human protein aggregate diseases caused by mutations in different proteins, both in the central nervous system and in striated muscle. The functional relationship between the expression of a mutated protein and mitochondrial dysfunction is largely unknown. In particular, the mechanism how this dysfunction drives the disease process is still...
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