Heterozygous P0 knockout mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP)

Michael E. Shy*, Edgardo Arroyo, John Sladky, Daniela Menichella, Huiyuan Jiang, Wenbo Xu, John Kamholz, Steven S. Scherer

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

98 Scopus citations

Abstract

Demyelinating peripheral neuropathies are clinically divided into inherited and acquired types. Inherited demyelinating neuropathies are caused by mutations in genes expressed by myelinating Schwann cells, whereas acquired ones, including chronic inflammatory demyelinating polyneuropathy (CIDP), are probably caused by autoimmune mechanisms. We find that heterozygous P0 knockout (P0+/-) mice develop a neuropathy that resembles CIDP. By one year of age, P0 +/- mice develop severe, asymmetric slowing of motor nerves, with temporal dispersion or conduction block, which are features of acquired demyelinating neuropathies including CIDP. Moreover, morphological analysis of affected nerves reveals severe and selective demyelination of motor fibers, focal regions of demyelination, and inflammatory cells. These data suggest that immune-mediated mechanisms may contribute to the pathogenesis of the neuropathy in P0+/- mice.

Original languageEnglish (US)
Pages (from-to)811-821
Number of pages11
JournalJournal of neuropathology and experimental neurology
Volume56
Issue number7
DOIs
StatePublished - Jul 1997

Keywords

  • Axon-Schwann cell interactions
  • CMT
  • Cheroot Marie-Tooth disease
  • Conduction block
  • Demyelination
  • My elin
  • Schwann cells

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Fingerprint Dive into the research topics of 'Heterozygous P<sub>0</sub> knockout mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP)'. Together they form a unique fingerprint.

Cite this