Article
Preaged remodeling of myofibrillar cytoarchitecture in skeletal muscle expressing R349P mutant desmin.
Neurobiology of aging - 1 Oct 2017
Diermeier Stefanie, Buttgereit Andreas, Schürmann Sebastian, Winter Lilli, Xu Hongyang, Murphy Robyn M, Clemen Christoph S, Schröder Rolf, Friedrich Oliver
Abstract excerpt
The majority of hereditary and acquired myopathies are clinically characterized by progressive muscle weakness. We hypothesized that ongoing derangement of skeletal muscle cytoarchitecture at the single fiber level may precede and be responsible for the progressive muscle weakness. Here, we analyzed the effects of aging in wild-type (wt) and heterozygous (het) and homozygous (hom) R349P desmin knock-in mice. The...
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