Characterisation of autoantibodies to peripheral myelin protein 22 in patients with hereditary and acquired neuropathies

J Neuroimmunol. 2000 May 1;104(2):155-63. doi: 10.1016/s0165-5728(99)00250-7.


To investigate the possibility that an autoimmune mechanism may play a role in the hereditary neuropathy Charcot-Marie-Tooth type 1A (CMT1A), sera were analysed by Western blot for anti-peripheral myelin protein 22 (PMP22) autoantibodies. These sera were compared with sera from patients with CMT type 2 (CMT2), acquired peripheral neuropathies such as chronic inflammatory demyelinating neuropathy (CIDP), anti-MAG IgM neuropathy, Miller-Fisher syndrome (MFS), diabetic neuropathy and with control blood donors. Anti-PMP22 positive sera were detected in 70% of patients with CMT1 and unexpectedly in 60% of patients with CMT2. Interestingly, 44% of the patients with other peripheral neuropathies and 23% of the apparently healthy controls showed also anti-PMP22 antibody reactivity. Immunohistochemical analysis of the human anti-PMP22 antisera on healthy sural nerve sections and on PMP22-expressing COS cells revealed that these sera did not recognise endogenous PMP22. Our results indicate that anti-PMP22 autoantibodies are found in sera of patients with different types of peripheral neuropathies, but their role in the pathogenesis of these diseases remains to be determined.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Autoantibodies / blood*
  • Blotting, Western
  • Case-Control Studies
  • Charcot-Marie-Tooth Disease / immunology*
  • Diabetic Neuropathies / immunology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Miller Fisher Syndrome / immunology
  • Myelin Proteins / immunology*
  • Polyneuropathies / immunology*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / immunology
  • Recombinant Proteins / immunology


  • Autoantibodies
  • Myelin Proteins
  • PMP22 protein, human
  • Recombinant Proteins