FBXL5−/− mice failed to sense intracellular iron levels and died in utero at embryonic day 8.5 as a result of iron overload and subsequent oxidative stress [38], whereas HOIL-1−/− mice exhibited amylopectin-like deposits in the myocardium, pathogen-specific immunodeficiency [42]. Here, RBCK1 is linked to immunodeficiency disease.