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

Br J Haematol. 1994 May;87(1):24-30. doi: 10.1111/j.1365-2141.1994.tb04865.x.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antigens, CD / blood*
  • Antigens, CD / genetics
  • Antigens, Surface / analysis
  • Base Sequence
  • CD55 Antigens
  • Cell Line / cytology
  • Cell Line / immunology*
  • Cell Separation
  • Cell Transformation, Viral
  • Clone Cells / immunology
  • Complement Inactivator Proteins / analysis*
  • Female
  • Glycosylphosphatidylinositols / blood
  • Hemoglobinuria, Paroxysmal / immunology*
  • Human T-lymphotropic virus 1
  • Humans
  • Membrane Glycoproteins / blood*
  • Membrane Glycoproteins / genetics
  • Middle Aged
  • Molecular Sequence Data
  • RNA, Messenger / analysis
  • T-Lymphocytes, Helper-Inducer / immunology*

Substances

  • Antigens, CD
  • Antigens, Surface
  • CD55 Antigens
  • Complement Inactivator Proteins
  • Glycosylphosphatidylinositols
  • Membrane Glycoproteins
  • RNA, Messenger