Immunophenotypic analysis. (A) Absolute numbers of peripheral blood CD3^-CD56⁺ NK cells in patient no. 30, as compared with healthy family donors. (B) For fluorescence-activated cell sorting (FACS) analysis of NKT cells, 100 000 events were recorded for each sample. NKT cells were detected as Vα24⁺Vβ11⁺CD3⁺ or 6b11⁺CD3⁺ cells. A marked reduction in the numbers of NKT cells was found in AP-3-deficient patients (no. 29 and no. 30) as compared with a homozygous healthy family donor (no. 32). (C) Absolute numbers of NKT cells in peripheral blood of AP-3-deficient patients as compared with healthy family donors (2-sided Student t test for unequal variances using logarithmic values, P < .002). (A, C) Horizontal bars indicate median values.