Abnormal Activation of BMP Signaling Causes Myopathy in Null Mice

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New strategies for treating congenital muscular dystrophies are needed. Current treatments are limited and aim to prolong ambulation and survival. Since most of the genes responsible for congenital muscular dystrophies are still unknown, elucidation of these genes may provide new insights that can lead to novel treatments. Fibrillin-2 null mice are born with myopathy and contractures and demonstrate accumulation of white fat during the early postnatal period. Both the histological features of myopathy and the accumulation of fat are rescued by inhibiting BMP signaling. Results indicate that FBN2 is a candidate gene for congenital muscular dystrophy and that strategies aimed at inhibition of abnormal BMP signaling may be applicable to muscular dystrophies. Furthermore, results reveal the importance of extracellular control of BMP signaling in skeletal muscle.

Vyšlo v časopise: Abnormal Activation of BMP Signaling Causes Myopathy in Null Mice. PLoS Genet 11(6): e32767. doi:10.1371/journal.pgen.1005340
Kategorie: Research Article
prolekare.web.journal.doi_sk: https://doi.org/10.1371/journal.pgen.1005340

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