Paroxysmal nocturnal hemoglobinuria and decay-accelerating factor.
The blood cells in paroxysmal nocturnal hemoglobinuria (PNH) lack several proteins, including some that regulate the activation of complement on the cell surface. Decay-accelerating factor (DAF), the first such protein to be identified, is, like all the missing proteins, affixed to the membrane by a glycolipid anchor containing phosphotidylinositol, hexoses, and ethanolamine. The defect in PNH appears to be an inability to place or maintain proteins linked in this way on the cell surface.
Department of Medicine, Duke University Medical Center, Durham, North Carolina 27710.
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D001798:Blood Proteins / Q000172:deficiency*; D018958:CD55 Antigens; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood*; D006801:Humans; D008565:Membrane Proteins / Q000172:deficiency*
D001798:Blood Proteins; D018958:CD55 Antigens; D008565:Membrane Proteins