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 beta1 integrin, a component of laminin receptors, causes a severe neuropathy with impaired radial sorting of axons. beta1-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 alpha6beta4 integrin. These data suggest that PI 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.

Conditional disruption of beta 1 integrin in Schwann cells impedes interactions with axons / M. Feltri, D. Porta, S. Previtali, A. Nodari, B. Migliavacca, A. Cassetti, A. Littlewood-Evans, L. Reichardt, A. Messing, A. Quattrini, U. Mueller, L. Wrabetz. - In: THE JOURNAL OF CELL BIOLOGY. - ISSN 0021-9525. - 156:1(2002), pp. 199-209. [10.1083/jcb.200109021]

Conditional disruption of beta 1 integrin in Schwann cells impedes interactions with axons

M. Feltri
Primo
Investigation
;
2002

Abstract

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 beta1 integrin, a component of laminin receptors, causes a severe neuropathy with impaired radial sorting of axons. beta1-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 alpha6beta4 integrin. These data suggest that PI 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.
Axo-glial interactions; Congenital muscular dystrophy; Cre/loxP; Laminin; Peripheral nerve
Settore BIO/17 - Istologia
Settore BIO/13 - Biologia Applicata
2002
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/953711
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