Comi Cristoforo, Gaviani Paola, Leone Maurizio, Ferretti Massimo, Castelli Luca, Mesturini Riccardo, Ubezio Gianluca, Chiocchetti Annalisa, Osio Maurizio, Muscia Francesco, Bogliun Graziella, Corso Giovanni, Gavazzi Armando, Mariani Claudio, Cantello Roberto, Monaco Francesco, Dianzani Umberto
Interdisciplinary Research Center of Autoimmune Diseases (IRCAD) and Department of Medical Sciences, 'A. Avogadro' University of Eastern Piedmont, Maggiore Hospital, Novara, Italy.
J Peripher Nerv Syst. 2006 Mar;11(1):53-60. doi: 10.1111/j.1085-9489.2006.00063.x.
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.
Fas死亡受体由活化的淋巴细胞表达，并参与免疫反应的终止。其遗传性缺陷会导致自身免疫性淋巴增殖综合征。Fas功能受损也可能在其他自身免疫性疾病中起作用，如多发性硬化症和1型糖尿病。这项工作的目的是评估慢性炎症性脱髓鞘性多发性神经病（CIDP）患者T细胞中的Fas功能。我们评估了27例CIDP患者、12例急性炎症性脱髓鞘性多发性神经病（AIDP）患者和110例对照的T细胞系中Fas诱导的凋亡。CIDP患者的Fas功能低于AIDP患者和对照，而AIDP患者和对照之间未发现统计学上的显著差异。此外，病程为进行性的CIDP患者的Fas功能低于复发缓解型患者，有轴索性损伤的CIDP患者的Fas功能低于单纯脱髓鞘型患者。这些数据表明，Fas功能缺陷有利于CIDP的发展和侵袭性进展。
