In rare AR T+B+NK+ SCID patients has been identified a SOCE-CRAC defect, caused by mutations in genes encoding two highly conserved proteins: ORAI1 (subunit forming pores in CRAC; its name comes from ORAI, the three sisters of Greek mythology), and STIM1 (stromal interaction molecule-1; it is the sensor of Ca2+ levels in ER and the activator of ORAI1-CRAC). The gene discussed is ORAI1; the disease is severe combined immunodeficiency.