The octapeptide repeat PrP c region and cobalamindeficient polyneuropathy of the rat

Giuseppe Scalabrino, Elena Mutti, Daniela Veber, Virginia Rodriguez Menendez, Cristina Novembrino, Alberto Calligaro, Giovanni Tredici

Research output: Contribution to journalArticle

Abstract

Introduction: Cobalamin (Cbl) deficiency affects the peripheral nervous system (PNS) morphologically and functionally. We investigated whether the octapeptide repeat (OR) region of prion protein (PrP C) (which is claimed to have myelinotrophic properties) is involved in the pathogenesis of rat Cbldeficient (Cbl-D) polyneuropathy. Methods: We intracerebroventricularly administered antibodies (Abs) against the OR region (OR-Abs) to Cbl-D rats to prevent myelin damage and maximum nerve conduction velocity (MNCV) abnormalities, and PrPCs to normal rats to reproduce PNS Cbl-D-like lesions. We measured nerve PrP c levels and MNCV. Results: The OR-Abs normalized myelin ultrastructure, MNCV values, and tumor necrosis factor (TNF)-α levels in the sciatic and tibial nerves of Cbl-D rats. PrP c levels increased in Cbl-D nerves. The nerves of the PrPC-treated rats showed typical Cbl-D lesions, significantly decreased MNCV values, and significantly increased TNF-α levels. Conclusions: OR-Abs prevent the myelin damage caused by increased OR regions, and excess TNF-α is involved in the pathogenesis of Cbl-D polyneuropathy.

Original languageEnglish
Pages (from-to)957-967
Number of pages11
JournalMuscle and Nerve
Volume44
Issue number6
DOIs
Publication statusPublished - Dec 2011

Keywords

  • Cellular prion protein
  • Cobalamin deficiency
  • Nerve conduction velocity
  • Peripheral nervous system
  • PrP octapeptide repeat region

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)
  • Physiology

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    Scalabrino, G., Mutti, E., Veber, D., Menendez, V. R., Novembrino, C., Calligaro, A., & Tredici, G. (2011). The octapeptide repeat PrP c region and cobalamindeficient polyneuropathy of the rat. Muscle and Nerve, 44(6), 957-967. https://doi.org/10.1002/mus.22225