The diseases include SEC61A1-linked common variable immunodeficiency, neutropenia and tubulointerstitial kidney disease [164,165,166,167,168], SEC61B- and SEC63-linked polycystic liver disease [54,169,170,171,172,173,174,175,176,177], and TRAP-, as well as TRC35-, TRC40-, and CAML-linked congenital disorders of glycosylation [46,135,178]. The gene discussed is SEC61B; the disease is familial juvenile hyperuricemic nephropathy.