Overexpression of ErbB2 and ErbB3 receptors in Schwann cells of patients with Charcot-Marie-Tooth disease type 1A

Roberto Massa, Camilla Palumbo, Tiziana Cavallaro, Maria Beatrice Panico, Roberto Bei, Chiara Terracciano, Nicolò Rizzuto, Giorgio Bernardi, Andrea Modesti

Research output: Contribution to journalArticle

Abstract

Axon-derived neuregulins (NRGs) are a family of growth factors whose binding to ErbB tyrosine kinase receptors promotes the maturation, proliferation and survival of Schwann cells (SCs). Correct NRG/ ErbB signaling is essential for the homeostasis of axonal-glial complexes and seems to play a role in peripheral nerve repair. The potential involvement of ErbB receptors in human peripheral neuropathies has not been clarified. Therefore, we assessed the immunoreactivity for EGFR (ErbB1), ErbB2, and ErbB3 in nerve biopsies from patients with different forms of Charcot-Marie-Tooth disease, type 1, (CMT1), as compared to others with inflammatory neuropathies and controls. The most notable changes consisted in the overexpression of ErbB2 and ErbB3 by SCs of nerves from CMT1A patients. These findings are consistent with an impairment of SC differentiation and expand the molecular phenotype of CMT1 A. The upregulation of these receptors may play a role in the inhibition of myelination or in the promotion of recurrent demyelination and axonal damage.

Original languageEnglish
Pages (from-to)342-349
Number of pages8
JournalMuscle and Nerve
Volume33
Issue number3
DOIs
Publication statusPublished - Mar 2006

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Keywords

  • Cell differentiation
  • Charcot-Marie-Tooth disease type 1A
  • ErbB receptors
  • Immunohistochemistry
  • Schwann cells

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Massa, R., Palumbo, C., Cavallaro, T., Panico, M. B., Bei, R., Terracciano, C., Rizzuto, N., Bernardi, G., & Modesti, A. (2006). Overexpression of ErbB2 and ErbB3 receptors in Schwann cells of patients with Charcot-Marie-Tooth disease type 1A. Muscle and Nerve, 33(3), 342-349. https://doi.org/10.1002/mus.20460