NFASC and Alzheimer disease: The third form of AD NF-κB2 disorders is caused by heterozygosity for variants that truncate the ankyrin repeat domain of p100 (p.Q539*, p.R611*, and p.R635*), or just upstream (p.E418*), resulting in a gain of p52 activity (p52GOF) due to spontaneous nuclear translocation of the mutant proteins (Fig. 1 B) (80).