Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytosis
J. Clin. Invest. Kimberly A. Risma, et al. 116:182 doi:10.1172/JCI26217 [Go to this article.]

Figure 4
Expression of perforin with missense mutations in RBL-1 cells. (A) FC was performed after retroviral infection of RBL-1 cells with PRF1 cDNA with and without missense mutations. Three independent experiments were performed. The perforin MCF for each mutant protein was normalized to WT and expressed as a percentage of WT perforin MCF (100%). Error bars represent SEM. (B) Immunohistochemistry of perforin in RBL-1 cells expressing WT and mutant perforin proteins. RBL-1 cells were grown in slide chambers, and adherent cells were analyzed for perforin staining with the monoclonal (δG9) anti-perforin antibody. Anti-mouse IgG coupled to HRP was used as a secondary antibody. The resulting stain is brown, whereas hematoxylin was used as a blue counterstain. Magnification, ×40.