Macrophage-related demyelination in peripheral nerves of mice deficient in the gap junction protein connexin 32

Igor Kobsar; Mathias Mäurer; Thomas Ott; Rudolf Martini

doi:10.1016/s0304-3940(02)00015-0

Macrophage-related demyelination in peripheral nerves of mice deficient in the gap junction protein connexin 32

Neurosci Lett. 2002 Mar 1;320(1-2):17-20. doi: 10.1016/s0304-3940(02)00015-0.

Authors

Igor Kobsar¹, Mathias Mäurer, Thomas Ott, Rudolf Martini

Affiliation

¹ Department of Neurology, Section of Developmental Neurobiology, University of Wurzburg, Josef-Schneider-Strasse 11, D-97080 Wurzburg, Germany.

PMID: 11849753
DOI: 10.1016/s0304-3940(02)00015-0

Abstract

Mice deficient in the gap junction protein connexin 32 (Cx32) develop a slowly progressing demyelinating neuropathy, with enlarged periaxonal collars, abnormal non-compacted myelin domains and axonal sprouts. These mice serve as a model for the X-linked form of inherited demyelinating neuropathies in humans. Based on our previous findings that macrophages are involved in demyelination in other myelin mutants (i.e. mice heterozygously deficient in P0), we considered the possibility that macrophages might be also mediators of demyelination in Cx32-deficient mice. Indeed, we detected an age-related increase in the number of macrophages in demyelinating nerves of Cx32-deficient mice. In addition, immunoelectron microscopy revealed macrophages in an apposition to degenerating myelin reminiscent of a macrophage-mediated demyelinating neuropathy. We conclude that involvement of macrophages might be a widespread phenomenon in genetically-determined demyelination.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Animals
Antigens, Differentiation / metabolism
Axons / metabolism
Axons / pathology
Axons / ultrastructure
Cell Count
Connexins / deficiency*
Connexins / genetics
Female
Gap Junction beta-1 Protein
Gap Junctions / genetics*
Gap Junctions / pathology
Gap Junctions / ultrastructure
Immunohistochemistry
Macrophages / metabolism*
Macrophages / pathology
Macrophages / ultrastructure
Male
Mice
Mice, Knockout
Microscopy, Electron
Myelin P0 Protein / deficiency
Myelin P0 Protein / genetics
Peripheral Nerves / metabolism*
Peripheral Nerves / pathology
Peripheral Nerves / ultrastructure
Polyradiculoneuropathy / genetics*
Polyradiculoneuropathy / immunology
Polyradiculoneuropathy / pathology
Schwann Cells / metabolism
Schwann Cells / pathology
Schwann Cells / ultrastructure
Up-Regulation / genetics
Up-Regulation / immunology

Substances

Antigens, Differentiation
Connexins
Myelin P0 Protein
monocyte-macrophage differentiation antigen