Genetic disorders of programmed cell death in the immune system.
摘要：
Human genetics offers new possibilities for understanding physiological regulatory mechanisms and disorders of the immune system. Genetic abnormalities of lymphocyte cell death programs have provided insights into mechanisms of receptor biology and principles of immune homeostasis and tolerance. Thus far, there are two major diseases of programmed cell death associated with inherited human mutations: the autoimmune lymphoproliferative syndrome and the caspase-eight deficiency state. We describe the details of their molecular pathogenesis and discuss how these diseases illustrate important concepts in immune regulation and genetics.
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关键词：
Humans Autoimmune Diseases Lymphoproliferative Disorders Caspases Antigens, CD95 Apoptosis Caspase 8 Caspase 10 Models, Immunological
DOI：
10.1146/annurev.immunol.24.021605.090513
被引量：
年份：
2006
国家科技图书文献中心
(权威机构)
Semantic Scholar
万方医学
掌桥科研
dx.doi.org
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