NDLI: BMP antagonists enhance myogenic differentiation and ameliorate the dystrophic phenotype in a DMD mouse model

Content Provider	PubMed Central
Author	Shi, Songting M.h., Hoogaars Willem Gorter, David J. J. De Van, Heiningen Sandra H. Lin, Herbert Y. Hong, Charles C. Kemaladewi, Dwi U. Aartsma-rus, Annemieke Dijke, Peter Ten ’t Hoen, Peter A.C.
Abstract	Duchenne Muscular Dystrophy (DMD) is an X-linked lethal muscle wasting disease characterized by muscle fiber degeneration and necrosis. The progressive pathology of DMD can be explained by an insufficient regenerative response resulting in fibrosis and adipose tissue formation. BMPs are known to inhibit myogenic differentiation and in a previous study we found increased expression of the BMP family member BMP4 in DMD myoblasts. The aim of the current study was therefore to investigate whether inhibition of BMP signaling could be beneficial for myoblast differentiation and muscle regeneration processes in a DMD context. All tested BMP inhibitors, Noggin, dorsomorphin and LDN-193189, were able to accelerate and enhance myogenic differentiation. However, dorsomorphin repressed both BMP and TGFβ signaling and was found to be toxic to primary myoblast cell cultures. In contrast, Noggin was found to be a potent and selective BMP inhibitor and was therefore tested in vivo in a DMD mouse model. Local adenoviral-mediated overexpression of Noggin in muscle resulted in increased expression of the myogenic regulatory genes Myog and Myod1 and improved muscle histology. In conclusion, our results suggest that repression of BMP signaling may constitute an attractive adjunctive therapy for DMD patients.
Related Links	http://dx.doi.org/10.1016/j.nbd.2010.10.003
Ending Page	360
Page Count	8
Starting Page	353
File Format	PDF
ISSN	09699961
e-ISSN	1095953X
Journal	Neurobiology of disease
Issue Number	2
Volume Number	41
Language	English
Publisher Date	2011-02-01
Access Restriction	Open
Subject Keyword	Neurology Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Neurology

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