自身免疫性淋巴增生综合征
细胞凋亡
生物
Fas配体
信号转导
Fas受体
程序性细胞死亡
细胞生物学
发病机制
自身免疫性疾病
免疫系统
免疫学
半胱氨酸蛋白酶
半胱氨酸蛋白酶8
遗传学
抗体
作者
Frédéric Rieux-Laucat,Alain Fischer,Françoise Le Deist
标识
DOI:10.1016/s0952-7915(03)00042-6
摘要
The autoimmune lymphoproliferative syndrome (ALPS) in humans and the lpr mouse strain are the first examples of primary apoptosis defects caused by inherited death-receptor mutations. They illustrate the role of Fas and Fas ligand in the control of autoimmune T-cell and B-cell proliferation. Subsequent analyses of ALPS in humans have highlighted the role of caspase 10 in the induction of apoptosis. The recent identification of a human caspase 8 defect has revealed a potential connection between apoptosis pathways and immune-receptor signaling. The genetic basis of ALPS is extremely complex, as multiple factors are potentially involved in the pathogenesis of this disease, thus offering an interesting model with which to unravel the mechanisms involved in T-cell homeostasis and self-tolerance.
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