title: Fas-mediated T-cell apoptosis is impaired in patients with chronic inflammatory demyelinating polyneuropathy.
abstract: The Fas death receptor is expressed by activated lymphocytes and is involved in switching-off the immune response. Its inherited defects cause auto-immune lymphoproliferative syndrome. Impaired Fas function may also play a role in other auto-immune diseases, such as multiple sclerosis and type 1 diabetes mellitus. The aim of this work was to evaluate Fas function in T cells from patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We evaluated Fas-induced apoptosis in T-cell lines from 27 patients with CIDP, 12 patients with acute inflammatory demyelinating polyneuropathy (AIDP), and 110 controls. CIDP patients displayed lower Fas function than both AIDP patients and controls, whereas no statistically significant difference was found between AIDP patients and controls. Moreover, Fas function was lower in CIDP patients with progressive course than in those with relapsing-remitting course and lower in CIDP patients with axonal damage than in those with pure demyelination. These data suggest that defective Fas function favours CIDP development and aggressive evolution.
authors.affiliation: Interdisciplinary Research Center of Autoimmune Diseases (IRCAD) and Department of Medical Sciences, 'A. Avogadro' University of Eastern Piedmont, Maggiore Hospital, Novara, Italy. comi@med.unipmn.it
authors.forename: Cristoforo
authors.initials: C
authors.lastname: Comi
authors.forename: Paola
authors.initials: P
authors.lastname: Gaviani
authors.forename: Maurizio
authors.initials: M
authors.lastname: Leone
authors.forename: Massimo
authors.initials: M
authors.lastname: Ferretti
authors.forename: Luca
authors.initials: L
authors.lastname: Castelli
authors.forename: Riccardo
authors.initials: R
authors.lastname: Mesturini
authors.forename: Gianluca
authors.initials: G
authors.lastname: Ubezio
authors.forename: Annalisa
authors.initials: A
authors.lastname: Chiocchetti
authors.forename: Maurizio
authors.initials: M
authors.lastname: Osio
authors.forename: Francesco
authors.initials: F
authors.lastname: Muscia
authors.forename: Graziella
authors.initials: G
authors.lastname: Bogliun
authors.forename: Giovanni
authors.initials: G
authors.lastname: Corso
authors.forename: Armando
authors.initials: A
authors.lastname: Gavazzi
authors.forename: Claudio
authors.initials: C
authors.lastname: Mariani
authors.forename: Roberto
authors.initials: R
authors.lastname: Cantello
authors.forename: Francesco
authors.initials: F
authors.lastname: Monaco
authors.forename: Umberto
authors.initials: U
authors.lastname: Dianzani
mesh_terms: D000328:Adult; D000368:Aged; D017209:Apoptosis / Q000502:physiology*; D015153:Blotting, Western; D053181:Caspase 8; D053453:Caspase 9; D020169:Caspases / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000276:immunology* / Q000378:metabolism / Q000503:physiopathology*; D018124:Receptors, Tumor Necrosis Factor / Q000378:metabolism*; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*; D019014:fas Receptor
chemical_list: C491901:FAS protein, human; D018124:Receptors, Tumor Necrosis Factor; D019014:fas Receptor; C505530:CASP8 protein, human; C506433:CASP9 protein, human; D053181:Caspase 8; D053453:Caspase 9; D020169:Caspases