A. FACS analysis of the GPI-anchored proteins CD16, FLAER,
CD66b and the combined measurement of CD55 and CD59 on neutrophils reveals a
significant reduction in mean fluorescence intensity (MFI) for patients 1 and 2
compared to control. B. MFI of CD59 on patient fibroblasts,
measured by immunofluorescence confocal microscopy, was less than 20% of the MFI
of the adult control (C1). C2 is a pediatric control that had lower CD59
expression C. MFI of CD87 (uPAR) on patient fibroblasts, measured
by immunofluorescence confocal microscopy, was less than 25% of the MFI of an
adult control (C1). C2 is a pediatric control that had lower CD87
expression.