Importantly, a patient with the Leu72→Pro missense mutation in the HOIP PUB domain reportedly exhibited multiorgan autoinflammation, immunodeficiency, amylopectinosis, and lymphangiectasia with impaired distributions and functions of T lymphocytes (41), suggesting that the effects of the mutation may be due to the dysfunctional binding of LUBAC to the CBM complex. The gene discussed is RNF31; the disease is Immunodeficiency.