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A correction to this article has been published: Suelves et al., J. Cell Biol. 179 (1) 165
Published online
doi:10.1083/jcb.200705127
The Journal of Cell Biology, Vol. 178, No. 6, 1039-1051
The Rockefeller University Press, 0021-9525 $30.00
© Suelves et al.
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Article

uPA deficiency exacerbates muscular dystrophy in MDX mice



Mònica Suelves1, Berta Vidal1,3, Antonio L. Serrano1,3, Marc Tjwa4, Josep Roma4, Roser López-Alemany8, Aernout Luttun5, María Martínez de Lagrán2,6, Maria Àngels Díaz8, Mercè Jardí1,3, Manuel Roig7, Mara Dierssen2,6, Mieke Dewerchin4, Peter Carmeliet4, and Pura Muñoz-Cánoves1,3

1 Program on Differentiation and Cancer and 2 Program on Genes and Disease, Center for Genomic Regulation, E-08003 Barcelona, Spain
3 Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas, E-08003 Barcelona, Spain
4 Center for Transgene Technology and Gene Therapy, Flanders Institute for Biotechnology, and 5 Center for Molecular and Vascular Biology, Katholieke Universiteit Leuven, Leuven B-3000, Belgium
6 Centro de Investigación Biomédica en Red de Enfermedades Raras, E-08003 Barcelona, Spain
7 Grup de Recerca de Malalties Neuro-metabòliques, Hospital Universitari Vall d'Hebron, E-08035 Barcelona, Spain
8 Institut d'Investigació Biomèdica de Bellvitge, E-08907 Barcelona, Spain

Correspondence to Pura Muñoz-Cánoves: pura.munoz{at}crg.es

Duchenne muscular dystrophy (DMD) is a fatal and incurable muscle degenerative disorder. We identify a function of the protease urokinase plasminogen activator (uPA) in mdx mice, a mouse model of DMD. The expression of uPA is induced in mdx dystrophic muscle, and the genetic loss of uPA in mdx mice exacerbated muscle dystrophy and reduced muscular function. Bone marrow (BM) transplantation experiments revealed a critical function for BM-derived uPA in mdx muscle repair via three mechanisms: (1) by promoting the infiltration of BM-derived inflammatory cells; (2) by preventing the excessive deposition of fibrin; and (3) by promoting myoblast migration. Interestingly, genetic loss of the uPA receptor in mdx mice did not exacerbate muscular dystrophy in mdx mice, suggesting that uPA exerts its effects independently of its receptor. These findings underscore the importance of uPA in muscular dystrophy.

Abbreviations used in this paper: BM, bone marrow; CK, creatine kinase; CTX, cardiotoxin; DMD, Duchenne muscular dystrophy; HE, hematoxylin/eosin; HGF, hepatocyte growth factor; SC, satellite cell; SF, scatter factor; uPA, urokinase plasminogen activator; uPAR, uPA receptor; WT, wild type.


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