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¹ Departments of Biological and Technological Research and Neuroscience, San Raffaele Scientific Institute, 20132 Milan, Italy
² Friedrich Miescher Institute, CH 4058 Basel, Switzerland
³ Department of Physiology, Howard Hughes Medical Institute, University of California, San Francisco, CA 94143
⁴ Waisman Center and School of Veterinary Medicine, University of Wisconsin, Madison, WI 53705

Address correspondence to Maria Laura Feltri, Department of Biological and Technological Research, San Raffaele Scientific Institute, Via Olgettina 58, 20132 Milan, Italy. Tel.: (39) 02-26434782 Fax: (39) 02-26434767. E-mail: feltri.laura{at}hsr.it

In dystrophic mice, a model of merosin-deficient congenital muscular dystrophy, laminin-2 mutations produce peripheral nerve dysmyelination and render Schwann cells unable to sort bundles of axons. The laminin receptor and the mechanism through which dysmyelination and impaired sorting occur are unknown. We describe mice in which Schwann cell–specific disruption of ß1 integrin, a component of laminin receptors, causes a severe neuropathy with impaired radial sorting of axons. ß1-null Schwann cells populate nerves, proliferate, and survive normally, but do not extend or maintain normal processes around axons. Interestingly, some Schwann cells surpass this problem to form normal myelin, possibly due to the presence of other laminin receptors such as dystroglycan and 6ß4 integrin. These data suggest that ß1 integrin links laminin in the basal lamina to the cytoskeleton in order for Schwann cells to ensheath axons, and alteration of this linkage contributes to the peripheral neuropathy of congenital muscular dystrophy.

Key Words: axo–glial interactions; Cre/loxP; congenital muscular dystrophy; laminin; peripheral nerve

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