Recessive STIM1 and ORAI1 loss-of-function (LoF) mutations inhibit SOCE and Ca2+ store refill and cause immunodeficiency (IMD9 and IMD10, OMIM # 612782, #612783), characterized by recurrent and chronic infections, autoimmunity, muscular hypotonia, mydriasis, and amelogenesis imperfecta [1,7,8]. This evidence concerns the gene ORAI1 and Immunodeficiency.