Immunological aspects of immunodeficiency diseases part 106

Immune 3



CD59 and CD55 are cell surface molecules anchored by glycosylphosphatidylinositol. These two proteins inactivate any C3 convertase molecules deposited on cell surfaces. Somatic mutation of the enzyme (PIGA: Phosphatidylinositol glican class A), needed to generate phospatidylinositol anchors for cell-surface proteins, including CD55 and CD59, in erythroid precursor, results in a condition called paroxysmal nocturnal hemoglobinuria, which is due to the increased susceptibility of red cells to complement-mediated hemolysis. Isolated CD59 deficiency also results in hemolytic anemia.

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