IKBKG mutations of the patients of this cohort and outcome after HSCT. (A) Schematic representation of the NEMO protein, with the various locations of the mutations found in the patients indicated. Patients with mutations affecting the zinc finger domain are shown in bold. The genetic defect of patient 26 (duplication of IKBKG intron 3 to exon 6) is not shown. (B) Overall survival rate of the 29 NEMO-deficient patients after HSCT. CC1, coiled coil domain 1; CC2, coiled coil domain 2; LZ, leucine zipper domain; NUB, NEMO ubiquitin-binding domain.