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The Role of Macrophages in Demyelinating Peripheral Nervous System of Mice Heterozygously Deficient in P0

By Stefano Carenini, Mathias Mäurer, Alexander Werner, Heinrich Blazyca, Klaus V. Toyka, Christoph D. Schmid, Gennadij Raivich and Rudolf Martini


Mice heterozygously deficient in the p0 gene (P0+/−) are animal models for some forms of inherited neuropathies. They display a progressive demyelinating phenotype in motor nerves, accompanied by mild infiltration of lymphocytes and increase in macrophages. We have shown previously that the T lymphocytes are instrumental in the demyelination process. This study addresses the functional role of the macrophage in this monogenic myelin disorder

Topics: Original Article
Publisher: The Rockefeller University Press
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Provided by: PubMed Central

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  5. (1997). Biology and action of colony-stimulating factor-1.
  6. (1995). Biology and genetics of hereditary motor and sensory neuropathies.
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  8. (1995). Charcot-Marie-Tooth disease type 1A: morphological phenotype of the 17p duplication versus PMP22 point mutations.
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  11. (1993). Coexistence of hereditary motor and sensory neuropathy type Ia and IgM paraproteinemic neuropathy.
  12. (1998). Connexin32-null mice develop demyelinating peripheral neuropathy.
  13. (1991). Corticosteroid-responsive dominantly inherited neuropathy in childhood.
  14. (1969). Electron microscope observations on demyelination and remyelination in experimental allergic neuritis.
  15. (1974). Genetic and clinical aspects of Charcot-Marie-Tooth’s disease.
  16. (1998). Guillain-Barre syndrome, CIDP and other chronic immune-mediated neuropathies.
  17. (1997). Heterozygous P0 knock-out mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP).
  18. (1995). HLA-DR expression in peripheral neuropathies: the role of Schwann cells, resident and hematogenous macrophages, and endoneurial fibroblasts.
  19. (1998). Human autoimmune neuropathies.
  20. (1993). Identification of macrophages and dendritic cells in the osteopetrotic (op/op) mouse.
  21. (2000). Immune deficiency in mouse models for inherited peripheral neuropathies leads to improved myelin maintenance.
  22. (1990). Impairment of macrophage colony-stimulating factor production and lack of resident bone marrow macrophages in the osteopetrotic op/op
  23. (1996). Inflammatory infiltrates in sural nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve.
  24. (1994). Inhibition of posttraumatic microglial proliferation in a genetic model of macrophage colony-stimulating factor deficiency in the mouse.
  25. (1999). Integrin family of cell adhesion molecules in the injured brain: regulation and cellular localization in the normal and regenerating mouse facial motor nucleus.
  26. (1998). Macrophage differentiation antigens in acute and chronic autoimmune polyneuropathies.
  27. (1998). Major histocompatibility complex class II expression and macrophage responses in genetically proven Charcot-Marie-Tooth type 1 and hereditary neuropathy with liability to pressure palsies.
  28. (1969). Mechanism of demyelination in experimental allergic neuritis. Electron microscopic studies.
  29. (1999). Mechanisms of immune regulation in the peripheral nervous system. Brain Pathol.
  30. (1991). Morphometrical and ultrastructural evaluation of the sural nerve in children with Charcot-Marie-Tooth: implications for pathogenesis and treatment.
  31. (1999). Mutations in the peripheral myelin genes and associated genes in inherited peripheral neuropathies.
  32. (2000). N-myc downstream-regulated gene 1 is mutated in hereditary motor and sensory neuropathy-Lom.
  33. (1992). Occurrence of active demyelinating lesions in children with hereditary motor and sensory neuropathy (HMSN) type I. Acta Neuropathol.
  34. (1996). Onion bulb cells in mice deficient for myelin genes share molecular properties with immature, differentiated non-myelinating and denervated Schwann cells.
  35. (1999). Overview of Charcot-Marie-Tooth disease 1A.
  36. (1995). P0-deficient mice show myelin degeneration in peripheral nerves characteristic of inherited human neuropathies.
  37. (1999). Phagocytosis of myelin in demyelinating disease: a review.
  38. (1982). Prednisone-responsive hereditary motor and sensory neuropathy. Mayo Clin.
  39. (1998). Regulation of MCSF receptors on microglia in the normal and injured mouse central nervous system: a quantitative immunofluorescence study using confocal laser microscopy.
  40. (1993). Selective elimination of macrophages by dichlormethylene diphosphonate-containing liposomes suppresses experimental autoimmune neuritis.
  41. (1997). Structural abnormalities and deficient maintenance of peripheral nerve myelin in mice lacking the gap junction protein connexin 32.
  42. (1984). The effect of suppression of macrophage activity on the development of experimental allergic neuritis.
  43. (1991). The Florey Lecture,
  44. (1995). The hematopoietic cytokine, colony-stimulating factor 1, is also a growth factor in the CNS: con-The
  45. (1990). The murine mutation osteopetrosis is in the coding region of the macrophage colony stimulating factor gene.

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