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. Here, RNF31 is linked to immunodeficiency disease.