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

J Neurosci Res. 2005 Mar 15;79(6):825-35. doi: 10.1002/jnr.20406.

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.

Publication types

  • Comparative Study

MeSH terms

  • Animals
  • Blotting, Northern / methods
  • Blotting, Western / methods
  • Charcot-Marie-Tooth Disease / metabolism*
  • Ciliary Neurotrophic Factor / genetics
  • Ciliary Neurotrophic Factor / metabolism
  • Connexins / genetics
  • Connexins / metabolism
  • Disease Models, Animal
  • Gap Junction beta-1 Protein
  • Gene Expression Regulation / genetics
  • Gene Expression Regulation / physiology*
  • Intracellular Signaling Peptides and Proteins / genetics
  • Intracellular Signaling Peptides and Proteins / metabolism
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Myelin Proteins / genetics
  • Myelin Proteins / metabolism
  • Myelin Proteolipid Protein / genetics
  • Myelin Proteolipid Protein / metabolism
  • Nerve Growth Factors
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism
  • RNA, Messenger / metabolism
  • Reverse Transcriptase Polymerase Chain Reaction / methods
  • S100 Calcium Binding Protein beta Subunit
  • S100 Proteins / genetics
  • S100 Proteins / metabolism
  • Sciatic Nerve / metabolism*
  • Sciatic Nerve / pathology

Substances

  • Ciliary Neurotrophic Factor
  • Connexins
  • Intracellular Signaling Peptides and Proteins
  • Myelin Proteins
  • Myelin Proteolipid Protein
  • Nerve Growth Factors
  • Nerve Tissue Proteins
  • Plp1 protein, mouse
  • Pmp22 protein, mouse
  • RNA, Messenger
  • S100 Calcium Binding Protein beta Subunit
  • S100 Proteins