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Human Molecular Genetics Advance Access originally published online on August 7, 2006
Human Molecular Genetics 2006 15(18):2690-2700; doi:10.1093/hmg/ddl201
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© The Author 2006. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Laminin {alpha}1 chain improves laminin {alpha}2 chain deficient peripheral neuropathy

Kinga I. Gawlik1, Jia-Yi Li2, Åsa Petersén2 and Madeleine Durbeej1,*

1 Muscle Biology Unit, Division for Cell and Matrix Biology and 2 Neuronal Survival Unit, Department of Experimental Medical Science, University of Lund, Lund, Sweden

* To whom correspondence should be addressed at: Division for Cell and Matrix Biology, Department of Experimental Medical Science, University of Lund, BMC B12, 221 84 Lund, Sweden. Tel: +46 462220812; Fax: +46 462220855; Email: madeleine.durbeej-hjalt{at}med.lu.se

Received June 15, 2006; Accepted July 27, 2006

Absence of laminin {alpha}2 chain leads to a severe form of congenital muscular dystrophy (MDC1A) associated with peripheral neuropathy. Hence, future therapies should be aimed at alleviating both muscle and neurological dysfunctions. Pre-clinical studies in animal models have mainly focused on ameliorating the muscle phenotype. Here we show that transgenic expression of laminin {alpha}1 chain in muscles and the peripheral nervous system of laminin {alpha}2 chain deficient mice reduced muscular dystrophy and largely corrected the peripheral nerve defects. The presence of laminin {alpha}1 chain in the peripheral nervous system resulted in near-normal myelination, restored Schwann cell basement membranes and improved rotarod performance. In summary, we postulate that laminin {alpha}1 chain is an excellent substitute for laminin {alpha}2 chain in multiple tissues and suggest that treatment with laminin {alpha}1 chain may be beneficial for MDC1A in humans.


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