Research ArticleMuscular Dystrophy

A Wnt-TGFβ2 axis induces a fibrogenic program in muscle stem cells from dystrophic mice

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Science Translational Medicine  17 Dec 2014:
Vol. 6, Issue 267, pp. 267ra176
DOI: 10.1126/scitranslmed.3008411

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Elucidating Satellite Stem Cell Biology

In patients affected by the muscle-wasting disease, Duchenne muscular dystrophy (DMD), the regenerative ability of skeletal muscle becomes progressively impaired, and fibrotic tissue accumulates, leading to a severe reduction in muscle function. Biressi et al. now show that satellite cells, the stem cells resident in skeletal muscle, have a compromised myogenic potential in the mdx mouse model of DMD. These muscle stem cells show enhanced expression of fibrogenic rather than myogenic genes. The authors link enhanced Wnt signaling with an increase in TGFβ2 expression in dystrophic muscle and suggest that the resulting increase in TGFβ activity may be responsible for the aberrant behavior of the satellite stem cells.

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