Reconstitution of CrkL restores NK cell function in patients with pDGS. PBMCs from 7 patients with pDGS were transduced with GFP (Control virus) or CrkL-GFP (CrkL virus) lentivirus. A, MFI of perforin and granzyme B in GFP+ NK cells in response to K562 stimulation. B, Percentage of IFN-γ+ and TNF-α+ transduced NK cells in response to phorbol 12-myristate 13-acetate and ionomycin stimulation. C and D, Quantification of CD107a total intensity on the plasma membrane. Transduced NK cells were activated on bilayers carrying anti-CD16–Alexa Fluor 568 and unlabeled ICAM-1 (Fig 6, C) or anti-NKG2D–Alexa Fluor 568 in the presence of unlabeled ICAM-1 and anti-2B4 (Fig 6, D). Data were pooled from 2 independent experiments. P values are for the paired t test (Fig 6, A and B) or the Student t test (Fig 6, C and D).