Article
Impaired growth and elevated fas receptor expression in PIGA(+) stem cells in primary paroxysmal nocturnal hemoglobinuria.
The Journal of clinical investigation - 1 Sept 2000
Chen R, Nagarajan S, Prince G M, Maheshwari U, Terstappen L W, Kaplan D R, Gerson S L, Albert J M, Dunn D E, Lazarus H M, Medof M E
Abstract excerpt
The genetic defect underlying paroxysmal nocturnal hemoglobinuria (PNH) has been shown to reside in PIGA, a gene that encodes an element required for the first step in glycophosphatidylinositol anchor assembly. Why PIGA-mutated cells are able to expand in PNH marrow, however, is as yet unclear. To address this question, we compared the growth of affected CD59(-)CD34(+) and unaffected CD59(+)CD34(+) cells from...
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