Interestingly, such diseases have not been reported in patients with AR RelB deficiency or IKK-α deficiency, suggesting that NIK plays a broader role in host immunity, potentially involving the canonical NF-κB pathway—either by directly regulating canonical NF-κB dimers or through the accumulation of unprocessed p100 (49, 50, 51). Here, NFKB2 is linked to hyperinsulinemic hypoglycemia, familial, 4.