Indeed, Orai1 as the pore-forming SOC unit was discovered throughout a genetic analysis of patients with autosomal recessive loss-of-function (LOF) or gain-of-function (GOF) mutations in ORAI1, which are associated with severe combined immunodeficiency (SCID)-like disease, tubular aggregate myopathy (TAM), and Stormorken syndrome (Feske, 2019). This evidence concerns the gene UBXN11 and severe combined immunodeficiency.