Journal Article

Laminin α1 chain improves laminin α2 chain deficient peripheral neuropathy

Kinga I. Gawlik, Jia-Yi Li, Åsa Petersén and Madeleine Durbeej

in Human Molecular Genetics

Volume 15, issue 18, pages 2690-2700
Published in print September 2006 | ISSN: 0964-6906
Published online August 2006 | e-ISSN: 1460-2083 | DOI:
Laminin α1 chain improves laminin α2 chain deficient peripheral neuropathy

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Absence of laminin α2 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 α1 chain in muscles and the peripheral nervous system of laminin α2 chain deficient mice reduced muscular dystrophy and largely corrected the peripheral nerve defects. The presence of laminin α1 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 α1 chain is an excellent substitute for laminin α2 chain in multiple tissues and suggest that treatment with laminin α1 chain may be beneficial for MDC1A in humans.

Journal Article.  5144 words.  Illustrated.

Subjects: Genetics and Genomics

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