Article
The molecular basis of paroxysmal nocturnal hemoglobinuria.
Haematologica - 1 Jan 2000
Rosti V
Abstract excerpt
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disease characterized by chronic intravascular hemolysis, cytopenia due to bone marrow failure and increased tendency to thrombosis. All patients with PNH studied so far have a somatic mutation in an X-linked gene, called PIG-A (phosphatidyl inositol glycan complementation group A), which encodes for a protein involved in the biosynthesis of the...
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