Quantitative analysis of the expression of glycosylphosphatidylinositol-anchored proteins during the maturation of different hematopoietic cell compartments of normal bone marrow.

Background: Glycosylphosphatidylinositol-anchored proteins (GPI-AP) are a heterogeneous group of proteins deficiently expressed in patients with paroxysmal nocturnal hemoglobinuria. Up till now, no study has been reported in which the exact patterns of expression of a large number of GPI-AP are quantitatively evaluated in normal bone marrow (BM) cells classified according to their lineage and maturation stage.

Methods: In the present study, we have quantitatively analyzed the expression of eleven different GPI-AP (CD14, CD16, CD24, CD48, CD52, CD58, CD59, CD66b, CD87, CD109 and CD157) during maturation of the neutrophil, monocytic, erythroid, lymphoid, basophil and plasmacytoid dendritic cells (DC) lineages in normal BM as a frame of reference for the understanding of the abnormal patterns of expression of GPI-AP observed in the BM of PNH patients.

Results: Our results show that expression of most GPI-AP varies during normal BM maturation, different profiles being frequently observed depending on the cell lineage or the GPI-AP analyzed.

Conclusion: Overall, these results provide a detailed map GPI-AP expression during normal hematopoietic differentiation, which could serve as a basis for the identification and characterization of changes occurring in PNH.