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GPI-defective monocytes from paroxysmal nocturnal hemoglobinuria patients show impaired in vitro dendritic cell differentiation.

Publication ,  Journal Article
Ruggiero, G; Terrazzano, G; Becchimanzi, C; Sica, M; Andretta, C; Masci, AM; Racioppi, L; Rotoli, B; Zappacosta, S; Alfinito, F
Published in: J Leukoc Biol
September 2004

Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal, acquired hematopoietic disorder characterized by a phosphatidylinositol (PI) glycan-A gene mutation, which impairs the synthesis of the glycosyl-PI (GPI) anchor, thus causing the absence of all GPI-linked proteins on the membrane of the clonal-defective cells. The presence of a consistent GPI-defective monocyte compartment is a common feature in PNH patients. To investigate the functional behavior of this population, we analyzed its in vitro differentiation ability toward functional dendritic cells (DCs). Our data indicate that GPI-defective monocytes from PNH patients are unable to undergo full DC differentiation in vitro after granulocyte macrophage-colony stimulating factor and recombinant interleukin (IL)-4 treatment. In this context, the GPI-defective DC population shows mannose receptor expression, high levels of the CD86 molecule, and impaired CD1a up-regulation. The analysis of lipopolysaccharide and CD40-dependent, functional pathways in these DCs revealed a strong decrease in tumor necrosis factor alpha and IL-12 production. Finally, GPI-defective DCs showed a severe impairment in delivering accessory signals for T cell receptor-dependent T cell proliferation.

Duke Scholars

Published In

J Leukoc Biol

DOI

ISSN

0741-5400

Publication Date

September 2004

Volume

76

Issue

3

Start / End Page

634 / 640

Location

England

Related Subject Headings

  • Up-Regulation
  • Tumor Necrosis Factor-alpha
  • T-Lymphocytes
  • Receptors, Cell Surface
  • Mutation
  • Monocytes
  • Membrane Glycoproteins
  • Mannose-Binding Lectins
  • Mannose Receptor
  • Male
 

Citation

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Chicago
ICMJE
MLA
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Ruggiero, G., Terrazzano, G., Becchimanzi, C., Sica, M., Andretta, C., Masci, A. M., … Alfinito, F. (2004). GPI-defective monocytes from paroxysmal nocturnal hemoglobinuria patients show impaired in vitro dendritic cell differentiation. J Leukoc Biol, 76(3), 634–640. https://doi.org/10.1189/jlb.1203607
Ruggiero, Giuseppina, Giuseppe Terrazzano, Cristina Becchimanzi, Michela Sica, Claudia Andretta, Anna Maria Masci, Luigi Racioppi, Bruno Rotoli, Serafino Zappacosta, and Fiorella Alfinito. “GPI-defective monocytes from paroxysmal nocturnal hemoglobinuria patients show impaired in vitro dendritic cell differentiation.J Leukoc Biol 76, no. 3 (September 2004): 634–40. https://doi.org/10.1189/jlb.1203607.
Ruggiero G, Terrazzano G, Becchimanzi C, Sica M, Andretta C, Masci AM, et al. GPI-defective monocytes from paroxysmal nocturnal hemoglobinuria patients show impaired in vitro dendritic cell differentiation. J Leukoc Biol. 2004 Sep;76(3):634–40.
Ruggiero, Giuseppina, et al. “GPI-defective monocytes from paroxysmal nocturnal hemoglobinuria patients show impaired in vitro dendritic cell differentiation.J Leukoc Biol, vol. 76, no. 3, Sept. 2004, pp. 634–40. Pubmed, doi:10.1189/jlb.1203607.
Ruggiero G, Terrazzano G, Becchimanzi C, Sica M, Andretta C, Masci AM, Racioppi L, Rotoli B, Zappacosta S, Alfinito F. GPI-defective monocytes from paroxysmal nocturnal hemoglobinuria patients show impaired in vitro dendritic cell differentiation. J Leukoc Biol. 2004 Sep;76(3):634–640.

Published In

J Leukoc Biol

DOI

ISSN

0741-5400

Publication Date

September 2004

Volume

76

Issue

3

Start / End Page

634 / 640

Location

England

Related Subject Headings

  • Up-Regulation
  • Tumor Necrosis Factor-alpha
  • T-Lymphocytes
  • Receptors, Cell Surface
  • Mutation
  • Monocytes
  • Membrane Glycoproteins
  • Mannose-Binding Lectins
  • Mannose Receptor
  • Male