As highlighted above, human SEC61A1 haploinsufficiency has recently been demonstrated to cause a primary antibody deficiency through impaired ASC differentiation (39), whereas IRF4 haploinsufficiency has been linked to Whipple’s disease caused by the inability to control infection with the bacteria Tropheryma whipplei (66). The gene discussed is SEC61A1; the disease is agammaglobulinemia.