Article
Role of phosphatidylinositol-linked proteins in paroxysmal nocturnal hemoglobinuria pathogenesis.
Annual review of medicine - 1 Jan 1996
Kinoshita T, Inoue N, Takeda J
Abstract excerpt
Patients with paroxysmal nocturnal hemoglobinuria have one or more mutant hematopoietic stem cell clones deficient in glycosylphosphatidylinositol (GPI)-anchor synthesis owing to somatic mutations in the X-linked gene PIG-A. The progeny of mutant stem cells dominates the peripheral blood. The presence of a large number of GPI-anchor deficient, complement-sensitive erythrocytes leads to hemolytic anemia. The...
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