Fas-, ceramide-, and PDN-induced T-cell death in 10 ALD families.

Members of each family are marked with the indicated symbols and were evaluated in the same experiment (different from that shown in Figure1). Two or 3 normal controls were included in each experiment and are shown in lane C. Day-21 T cells were treated with the indicated reagent and survival was assessed after 18 hours. Results are expressed as specific cell survival percentage. The horizontal lines indicate the upper limit of the normal range calculated as the mean + 2 SD from data obtained from 75 normal donors. Among the close relatives, the aunt was selected because she displayed an ALPS-like clinical pattern, whereas the 3 healthy siblings were casually selected. All relatives analyzed are included in the text. C indicates normal controls; F, fathers; M, mothers; Pt, ALD patients; R, close relatives.