Pedigrees of patients ALPS-5 and -11 and DALD-3,-6, and -10. (A) Inheritance of the N252S PRF1 mutation (PRF1#) and defective Fas function (D) in ALPS-5 and -11; inheritance of the Fas mutation (TNFRSF6*) is also shown for ALPS-5. (B) Inheritance of the A91V PRF1 variation (PRF1°) and defective Fas function (D) in the 3 DALD patients. Subjects with ALPS/DALD are marked in gray; the sister of DALD-3 displayed CVID. PRF1°° marks A91V homozygotes. Fas function was evaluated as reported in Figure 1.