Skeletal muscle fibrosis develops in response to desmin deletion

GA Meyer, RL Lieber - American Journal of Physiology-Cell …, 2012 - journals.physiology.org
American Journal of Physiology-Cell Physiology, 2012journals.physiology.org
Skeletal muscle is a dynamic composite of proteins that responds to both internal and
external cues to facilitate muscle adaptation. In cases of disease or altered use, these
messages can be distorted resulting in myopathic conditions such as fibrosis. In this work,
we describe a mild and progressive fibrotic adaptation in skeletal muscle lacking the
cytoskeletal intermediate filament protein desmin. Muscles lacking desmin become
progressively stiffer, accumulate increased collagen, and increase expression of genes …
Skeletal muscle is a dynamic composite of proteins that responds to both internal and external cues to facilitate muscle adaptation. In cases of disease or altered use, these messages can be distorted resulting in myopathic conditions such as fibrosis. In this work, we describe a mild and progressive fibrotic adaptation in skeletal muscle lacking the cytoskeletal intermediate filament protein desmin. Muscles lacking desmin become progressively stiffer, accumulate increased collagen, and increase expression of genes involved in extracellular matrix turnover. Additionally, in the absence of desmin, skeletal muscle is in an increased state of inflammation and regeneration as indicated by increased centrally nucleated fibers, elevated inflammation and regeneration related gene expression, and increased numbers of inflammatory cells. These data suggest a potential link between increased cellular damage and the development of fibrosis in muscles lacking the cytoskeletal support of the desmin filament network.
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