Recessive STIM1 and ORAI1 loss-of-function (LoF) mutations resulting in insufficient SOCE cause CRAC channelopathies characterized by severe combined immunodeficiency (SCID) involving recurrent and chronic infections, autoimmunity, muscular hypotonia, ectodermal dysplasia, anhidrosis, and mydriasis (Feske et al., 2006; Picard et al., 2009; Lacruz and Feske, 2015). This evidence concerns the gene ORAI1 and severe combined immunodeficiency.