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Expression profiling of sciatic nerve in a Charcot-Marie-Tooth disease type 1a mouse model

✍ Scribed by Anneloor L.M.A. ten Asbroek; Camiel Verhamme; Marjon van Groenigen; Ruud Wolterman; Maryla M. de Kok-Nazaruk; Frank Baas

Publisher
John Wiley and Sons
Year
2005
Tongue
English
Weight
303 KB
Volume
79
Category
Article
ISSN
0360-4012

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✦ Synopsis

Abstract

Expression profiling was performed on sciatic nerve of normal mice and of transgenic mice overexpressing the peripheral myelin protein 22 kDa (PMP22). These mice represent a model for the hereditary peripheral neuropathy Charcot‐Marie Tooth type 1A. Comparison of the profiles reveals that the proteasomal degradation pathway and various signaling mechanisms are up‐regulated in the diseased nerve. The down‐regulated processes represent cell shape and adhesion as well as cellular activity and metabolism. In addition, we found that the most significantly up‐regulated differences could not be mapped on known transcripts and thus might represent not identified transcripts. Our data will be helpful to direct future research aimed at deciphering the molecular pathogenesis of the most prevalent hereditary peripheral neuropathy. © 2005 Wiley‐Liss, Inc.

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