PNH - 3
Defects cont’d:
Specific
proteins missing on PNH cells:
CD55:
Decay accelerating factor
Why
components are missing:
These
proteins, along with others, use a glycosylphosphotidylinositol
(GPI) anchor on membrane and this component is abnormal
in PNH patients because the gene PIGA is responsible for
correcting the abnormal (GPI).
CD59:
Membrane inhibitor of
reactive lysis