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J Cell Biol. 2007 Sep 10;178(6):1039-51. Epub 2007 Sep 4.

uPA deficiency exacerbates muscular dystrophy in MDX mice.

Author information

1
Program on Differentiation and Cancer, Center for Genomic Regulation, E-08003, Barcelona, Spain.

Erratum in

  • J Cell Biol. 2007 Oct 8;179(1):165. Díaz, Maria Angels [corrected to Díaz-Ramos, Angels].

Abstract

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.

PMID:
17785520
PMCID:
PMC2064626
DOI:
10.1083/jcb.200705127
[Indexed for MEDLINE]
Free PMC Article
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