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First published online 9 May 2006
doi: 10.1242/jcs.02952

Journal of Cell Science 119, 2185-2195 (2006)
Published by The Company of Biologists 2006
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Research Article

Severe muscular dystrophy in mice that lack dystrophin and {alpha}7 integrin

Jachinta E. Rooney1,*, Jennifer V. Welser1,*, Melissa A. Dechert1, Nichole L. Flintoff-Dye1, Stephen J. Kaufman2 and Dean J. Burkin1,3,{ddagger}

1 Department of Pharmacology, University of Nevada, Reno, NV 89557, USA
2 Department of Cell and Developmental Biology, University of Illinois, Urbana, IL 61801, USA
3 Nevada Transgenic Center, University of Nevada, Reno, NV 89557, USA

{ddagger} Author for correspondence (e-mail: dburkin{at}med.unr.edu)

Accepted 21 February 2006

The dystrophin glycoprotein complex links laminin in the extracellular matrix to the cell cytoskeleton. Loss of dystrophin causes Duchenne muscular dystrophy, the most common human X-chromosome-linked genetic disease. The {alpha}7ß1 integrin is a second transmembrane laminin receptor expressed in skeletal muscle. Mutations in the {alpha}7 integrin gene cause congenital myopathy in humans and mice. The {alpha}7ß1 integrin is increased in the skeletal muscle of Duchenne muscular dystrophy patients and mdx mice. This observation has led to the suggestion that dystrophin and {alpha}7ß1 integrin have complementary functional and structural roles. To test this hypothesis, we generated mice lacking both dystrophin and {alpha}7 integrin (mdx/{alpha}7-/-). The mdx/{alpha}7-/- mice developed early-onset muscular dystrophy and died at 2-4 weeks of age. Muscle fibers from mdx/{alpha}7-/- mice exhibited extensive loss of membrane integrity, increased centrally located nuclei and inflammatory cell infiltrate, greater necrosis and increased muscle degeneration compared to mdx or {alpha}7-integrin null animals. In addition, loss of dystrophin and/or {alpha}7 integrin resulted in altered expression of laminin-{alpha}2 chain. These results point to complementary roles for dystrophin and {alpha}7ß1 integrin in maintaining the functional integrity of skeletal muscle.

Key words: {alpha}7 integrin, Dystrophin, Transgenic mice, Double knockout, Skeletal muscle, Muscular dystrophy




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© The Company of Biologists Ltd 2006