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 2
Detection of mouse perforin expressed in RBL cells. Mouse perforin cDNA was introduced into the MIEG3 viral vector, and RBL-1 and RBL-2H3 cells were infected with retrovirus. Mouse perforin expressed in both cells underwent a maturation process that could be blocked by CMA. However, under nonreducing and reducing conditions, mouse perforin had a slower mobility than human and rat perforin expressed in both cells. For RBL-2H3 cells, 100 μg protein was loaded per lane, except for RBL-2H3 cells expressing rat perforin, which required only 10 μg protein to detect perforin. For RBL-1 cells, 10 μg protein was loaded per lane for all 3 cell lines. Detection was by P1-8 antibody.