Cellular mechanisms of tissue fibrosis. 4. Structural and functional consequences of skeletal muscle fibrosis

Lieber RL, Ward SR.
Am J Physiol Cell Physiol, 2013 305(3):C241-52. Epub 2013 Jun 12.


Skeletal muscle fibrosis can be a devastating clinical problem that arises from many causes, including primary skeletal muscle tissue diseases, as seen in the muscular dystrophies, or can be secondary to events that include trauma to muscle or brain injury. The cellular source of activated fibroblasts (myofibroblasts) may include resident fibroblasts, muscle adult stem cells, inflammatory or perivascular cells depending upon the model studied. Even though it is likely that there is no single source for the myofibroblasts, a common mechanism for the production of fibrosis is via the TGFΒ/PSmad3 pathway. This pathway and its downstream targets thus provide loci for anti-fibrotic therapies as do methods for blocking the transdifferentiation of progenitors into activated fibroblasts. A structural model for the extracellular collagen network of skeletal muscle is needed to facilitate measurements of collagen content, morphology and gene expression to be related to mechanical properties. Approaches used to study fibrosis in tissues, such as lung, kidney and liver, need to be applied to studies of skeletal muscle so as to identify ways to prevent or even cure the devastating maladies of skeletal muscle.

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