N-WASp is required for Schwann cell cytoskeletal dynamics, normal myelin gene expression and peripheral nerve myelination.

Published

Journal Article

Schwann cells elaborate myelin sheaths around axons by spirally wrapping and compacting their plasma membranes. Although actin remodeling plays a crucial role in this process, the effectors that modulate the Schwann cell cytoskeleton are poorly defined. Here, we show that the actin cytoskeletal regulator, neural Wiskott-Aldrich syndrome protein (N-WASp), is upregulated in myelinating Schwann cells coincident with myelin elaboration. When N-WASp is conditionally deleted in Schwann cells at the onset of myelination, the cells continue to ensheath axons but fail to extend processes circumferentially to elaborate myelin. Myelin-related gene expression is also severely reduced in the N-WASp-deficient cells and in vitro process and lamellipodia formation are disrupted. Although affected mice demonstrate obvious motor deficits these do not appear to progress, the mutant animals achieving normal body weights and living to advanced age. Our observations demonstrate that N-WASp plays an essential role in Schwann cell maturation and myelin formation.

Full Text

Cited Authors

  • Jin, F; Dong, B; Georgiou, J; Jiang, Q; Zhang, J; Bharioke, A; Qiu, F; Lommel, S; Feltri, ML; Wrabetz, L; Roder, JC; Eyer, J; Chen, X; Peterson, AC; Siminovitch, KA

Published Date

  • April 2011

Published In

Volume / Issue

  • 138 / 7

Start / End Page

  • 1329 - 1337

PubMed ID

  • 21385763

Pubmed Central ID

  • 21385763

Electronic International Standard Serial Number (EISSN)

  • 1477-9129

International Standard Serial Number (ISSN)

  • 0950-1991

Digital Object Identifier (DOI)

  • 10.1242/dev.058677

Language

  • eng