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Expression profiling of sciatic nerve in a Charcot‐Marie‐Tooth disease type 1a mouse model
Author(s) -
ten Asbroek Anneloor L.M.A.,
Verhamme Camiel,
van Groenigen Marjon,
Wolterman Ruud,
de KokNazaruk Maryla M.,
Baas Frank
Publication year - 2005
Publication title -
journal of neuroscience research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.72
H-Index - 160
eISSN - 1097-4547
pISSN - 0360-4012
DOI - 10.1002/jnr.20406
Subject(s) - pathogenesis , sciatic nerve , peripheral myelin protein 22 , myelin , peripheral neuropathy , genetically modified mouse , peripheral nerve , biology , pathology , microbiology and biotechnology , neuroscience , transgene , medicine , anatomy , gene , genetics , central nervous system , endocrinology , diabetes mellitus
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.