Expression profiling of sciatic nerve in CMT 1A mouse model

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Abstract from J Neurosci Res. 2005 Jan 25

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

mouse model.

Ten Asbroek AL, Verhamme C, van Groenigen M, Wolterman R, de Kok-Nazaruk MM,

Baas F.

Neurogenetics Laboratory, Academic Medical Center, Amsterdam, The Netherlands.

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.