Journal
JOURNAL OF PHYSIOLOGY-LONDON
Volume 590, Issue 17, Pages 4157-4167Publisher
WILEY
DOI: 10.1113/jphysiol.2012.233221
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Funding
- NIH [AI066128]
- National Multiple Sclerosis Society
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Store-operated Ca2+ entry (SOCE) in cells of the immune system is mediated by Ca2+ release-activated Ca2+ (CRAC) channels that are formed by ORAI1 and its homologues ORAI2 and ORAI3. They are activated by stromal interaction molecules (STIM) 1 and 2 in response to depletion of endoplasmic reticulum Ca2+ stores. Loss-of-function mutations in the human ORAI1 and STIM1 genes abolish CRAC channel function and SOCE in a variety of non-excitable cells including lymphocytes and other immune cells, resulting in a unique clinical syndrome termed CRAC channelopathy. It is dominated by severe immunodeficiency and autoimmunity due to impaired SOCE and defects in the function of several lymphocyte subsets. These include CD8+ T cells, CD4+ effector and regulatory T cells, natural killer (NK) cells and B cells. This review provides a concise discussion of the role of CRAC channels in these lymphocyte populations and the regulation of adaptive immune responses to infection, in autoimmunity and inflammation.
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