Human Molecular Genetics Advance Access published online on June 22, 2004
Human Molecular Genetics, doi:10.1093/hmg/ddh190
© 2004 by Oxford University Press
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1 Department of Cell and Molecular Biology, Section for Cell and Developmental Biology, University of Lund, Lund, Sweden
* To whom correspondence should be addressed. E-mail: madeleine.durbeej_hjalt{at}medkem.lu.se.
Laminin
Article
Laminin
1 chain reduces muscular dystrophy in laminin 2 chain deficient mice
2 Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
3 University of Lund, Department of Cell and Molecular Biology, Section for Cell and Developmental Biology, BMC B12, 221 84 Lund, Sweden
Abstract 
2 chain deficiency in humans and mice leads to severe forms of congenital muscular dystrophy. Here, we investigated whether laminin 1 chain in mice can compensate for the absence of laminin 2 chain and prevent the development of muscular dystrophy. We generated mice expressing a laminin 1 chain transgene in skeletal muscle of laminin 2 chain deficient mice. Laminin 1 is not normally expressed in muscle, but the transgenically produced laminin 1 chain was incorporated into muscle basement membranes, and normalized the compensatory changes of expression of certain other laminin chains (4, 
2). In 4-month-old mice, laminin 1 chain could fully prevent the development of muscular dystrophy in several muscles, and partially in others. The laminin 1 chain transgene not only reversed the appearance of histopathological features of the disease to a remarkable degree, but also greatly improved health and longevity of the mice. Correction of laminin 2 chain deficiency by laminin 1 chain may serve as a paradigm for gene therapy of congenital muscular dystrophy in patients.
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