MpzR98C arrests Schwann cell development in a mouse model of early-onset Charcot–Marie–Tooth disease type 1B

Mutations in myelin protein zero (MPZ) cause Charcot–Marie–Tooth disease type 1B. Many dominant MPZ mutations, including R98C, present as infantile onset dysmyelinating neuropathies. We have generated an R98C ‘knock-in’ mouse model of Charcot–Marie–Tooth type 1B, where a mutation encoding R98C was t...

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Hauptverfasser: Saporta, Mario A. C., Shy, Brian R., Patzko, Agnes, Bai, Yunhong, Pennuto, Maria, Ferri, Cinzia, Tinelli, Elisa, Saveri, Paola, Kirschner, Dan, Crowther, Michelle, Southwood, Cherie, Wu, Xingyao, Gow, Alexander, Feltri, M. Laura, Wrabetz, Lawrence, Shy, Michael E.
Format: Artigo
Sprache:Inglês
Veröffentlicht: Oxford University Press 2012
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Original Articles
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3381724/
https://ncbi.nlm.nih.gov/pubmed/22689911
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/brain/aws140
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