[Expression of GPI-anchor proteins during the differentiation from hematopoietic progenitors in patients with paroxysmal nocturnal hemoglobinuria].
We studied the expression of HRF20, one of the GPI-anchor proteins, on the hematopoietic progenitors and their progeny cells grown in cultures with bone marrow cells from patients with paroxysmal nocturnal hemoglobinuria (PNH). PNH bone marrow cells were sorted by fluorescence-activated cell sorter with anti-HRF20 monoclonal antibody and then cultured in methylcellulose. Every CFU-GM colonies and BFU-E bursts formed from the HRF20-negative fraction were HRF20-negative. On the other hand, there were HRF20-positive colonies and bursts as well as HRF20-negative ones in cultures with bone marrow cells from the HRF20-positive fraction. The results suggested that abnormal events can occur during the several steps of differentiation form PNH hematopoietic stem cells.
2nd Department of Internal Medicine, Hyogo College of Medicine.
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Kanamaru
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Nishimura
D001854:Bone Marrow Cells; D002454:Cell Differentiation; D002478:Cells, Cultured; D017261:Glycosylphosphatidylinositols / Q000172:deficiency / Q000378:metabolism*; D006412:Hematopoietic Stem Cells / Q000166:cytology*; D006457:Hemoglobinuria, Paroxysmal / Q000473:pathology*; D006801:Humans; D008562:Membrane Glycoproteins / Q000378:metabolism*
D017261:Glycosylphosphatidylinositols; D008562:Membrane Glycoproteins