Establishment of a human T-cell line with deficient surface expression of glycosylphosphatidylinositol (GPI)-anchored proteins from a patient with paroxysmal nocturnal haemoglobinuria.

A novel interleukin-2 dependent T-cell line, PMT-2Y, was established from the peripheral blood of a patient with paroxysmal nocturnal haemoglobinuria (PNH) by human T lymphotropic virus type I (HTLV-I)-mediated transformation. PMT-2Y cells are positive for CD2, CD3, CD4, CD25, T cell receptor alpha beta and HLA-DR, but negative for CD1, CD7, CD8, CD19 and CD20, indicating that the clone belongs to a helper/inducer subset of T cells. PMT-2Y cells have the monoclonal integration of HTLV-I proviral DNA, suggesting that they derived from a single clone. Moreover, they lack surface expression of complement regulatory proteins such as DAF (CD55) and CD59, that are the most important glycosylphosphatidylinositol (GPI)-anchored membrane proteins defective in haemopoietic cells of patients with PNH. Northern blot analysis, however, revealed the production of normal levels of DAF mRNAs. Thus, PMT-2Y is derived from a PNH T cell clone and may be a useful model to study PNH.