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.
Rosse
W F
WF
Department of Medicine, Duke University Medical Center, Durham, North Carolina 27710.
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