<prompt> Paroxysmal nocturnal hemoglobinuria results from acquired mutations inPML-RARABCL-2NOTCH1PIGA  <response>: Paroxysmal nocturnal hemoglobinuria PNH is a disease that results from acquired mutations in the phosphatidylinositol glycan complementation group A gene PIGA, an enzyme that is essential for the synthesis of ceain membrane-associated complement regulatory proteins.PNH blood cells are deficient in three GPI-linked proteins that regulate complement activity: 1 decay accelerating factor, or CD55; 2 membrane inhibitor of reactive lysis, or CD59; and 3 C8 binding protein.Of these factors, the most impoant is CD59, a potent inhibitor of C3 convease that prevents the spontaneous activation of the alternative complement pathway.Red cells deficient in these GPI-linked factors are abnormally susceptible to lysis or injury by complement and manifests as intravascular hemolysisRobbins pathology 9e pg: 642 