Article
T cells from paroxysmal nocturnal haemoglobinuria (PNH) patients show an altered CD40-dependent pathway.
Journal of leukocyte biology - 1 Jul 2005
Terrazzano Giuseppe, Sica Michela, Becchimanzi Cristina, Costantini Silvia, Rotoli Bruno, Zappacosta Serafino, Alfinito Fiorella, Ruggiero Giuseppina
Abstract excerpt
Paroxysmal nocturnal haemoglobinuria (PNH) is a haematopoiesis disorder characterized by the expansion of a stem cell bearing a somatic mutation in the phosphatidylinositol glycan-A (PIG-A) gene, which is involved in the biosynthesis of the glycosylphosphatidylinositol (GPI) anchor. A number of data suggest the inability of the PIG-A mutation to account alone for the clonal dominance of the GPI-defective clone...
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