title: Characterisation of autoantibodies to peripheral myelin protein 22 in patients with hereditary and acquired neuropathies.
abstract: 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.
authors.affiliation: Department of Research, University Hospital Basel, Switzerland.
authors.forename: M F
authors.initials: MF
authors.lastname: Ritz
authors.forename: J
authors.initials: J
authors.lastname: Lechner-Scott
authors.forename: R J
authors.initials: RJ
authors.lastname: Scott
authors.forename: P
authors.initials: P
authors.lastname: Fuhr
authors.forename: N
authors.initials: N
authors.lastname: Malik
authors.forename: B
authors.initials: B
authors.lastname: Erne
authors.forename: V
authors.initials: V
authors.lastname: Taylor
authors.forename: U
authors.initials: U
authors.lastname: Suter
authors.forename: N
authors.initials: N
authors.lastname: Schaeren-Wiemers
authors.forename: A J
authors.initials: AJ
authors.lastname: Steck
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D015153:Blotting, Western; D016022:Case-Control Studies; D002607:Charcot-Marie-Tooth Disease / Q000276:immunology*; D003929:Diabetic Neuropathies / Q000276:immunology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019846:Miller Fisher Syndrome / Q000276:immunology; D009185:Myelin Proteins / Q000276:immunology*; D011115:Polyneuropathies / Q000276:immunology*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000276:immunology; D011994:Recombinant Proteins / Q000276:immunology
chemical_list: D001323:Autoantibodies; D009185:Myelin Proteins; C073687:PMP22 protein, human; D011994:Recombinant Proteins