The differential responses of the patient's PBMCs are due to differential expression of NEMO in each lineage. (A) Defective response of the patient's PBMCs to LPS but not to IL-18. PBMCs from the patient and healthy controls were stimulated with varying doses of IL-18 plus IL-12 (20 ng/mL) or with varying doses of LPS plus IFN-γ (5000 U/mL). A representative of 2 consistent results is shown. (B) Western blotting analysis of NEMO. Fifty micrograms of PBMC cell lysates (lanes 1-3), HTLV-I-transformed cells (lanes 4-5), and EBV-transformed cells (lanes 6-7) from the patient (lanes 3, 5, and 7) and healthy controls (lanes 1, 2, 4, and 6) were used. β-actin was used as a control for equal loading (lanes 1-7).