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

Autor(es): Ritz M F,Lechner-Scott J,Scott R J,Fuhr P,Malik N,Erne B,Taylor V,Suter U,Schaeren-Wiemers N,Steck A J

Resumo: 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.

Palavras-Chave: Peripheral myelin protein 22; Autoantibody; Charcot-Marie-Tooth disease; Hereditary neuropathy; Acquired neuropathy

Imprenta: Journal of Neuroimmunology, v. 104, n. 2, p. 155-163, 2000

Identificador do objeto digital: 10.1016/S0165-5728(99)00250-7

Descritores: Guillain-Barre Syndrome - Cell ; Guillain-Barre Syndrome - Pathogenesis ; Guillain-Barre Syndrome - Proteins ; Guillain-Barre Syndrome - Antibodies ; Guillain-Barre Syndrome - Immunology ; Guillain-Barre Syndrome - Public health

Data de publicação: 2000