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Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity.

Publication ,  Journal Article
Nakajima, H; Cella, M; Bouchon, A; Grierson, HL; Lewis, J; Duckett, CS; Cohen, JI; Colonna, M
Published in: Eur J Immunol
November 2000

Patients with the X-linked lymphoproliferative disorder (XLPD) are unable to control Epstein-Barr virus (EBV)-induced infections and lymphoproliferation. This disease is caused by a deficit of SAP, an adapter protein involved in the signal transduction of several cell surface receptors of the CD2 superfamily. One of these receptors, called 2B4, is expressed on NK cells, cytotoxic T cells and myeloid cells and activates NK cell cytotoxicity. Here we show that XLPD patients have a defect of 2B4 receptor-mediated NK cell cytotoxicity. This defect may contribute to the pathogenesis of XLPD by reducing NK cell lysis of EBV-infected B cells.

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Published In

Eur J Immunol

DOI

ISSN

0014-2980

Publication Date

November 2000

Volume

30

Issue

11

Start / End Page

3309 / 3318

Location

Germany

Related Subject Headings

  • Signaling Lymphocytic Activation Molecule Family
  • Signaling Lymphocytic Activation Molecule Associated Protein
  • Signal Transduction
  • Receptors, Immunologic
  • Membrane Glycoproteins
  • Lymphoproliferative Disorders
  • Killer Cells, Natural
  • Intracellular Signaling Peptides and Proteins
  • Immunology
  • Humans
 

Citation

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Nakajima, H., Cella, M., Bouchon, A., Grierson, H. L., Lewis, J., Duckett, C. S., … Colonna, M. (2000). Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity. Eur J Immunol, 30(11), 3309–3318. https://doi.org/10.1002/1521-4141(200011)30:11<3309::AID-IMMU3309>3.0.CO;2-3
Nakajima, H., M. Cella, A. Bouchon, H. L. Grierson, J. Lewis, C. S. Duckett, J. I. Cohen, and M. Colonna. “Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity.Eur J Immunol 30, no. 11 (November 2000): 3309–18. https://doi.org/10.1002/1521-4141(200011)30:11<3309::AID-IMMU3309>3.0.CO;2-3.
Nakajima H, Cella M, Bouchon A, Grierson HL, Lewis J, Duckett CS, et al. Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity. Eur J Immunol. 2000 Nov;30(11):3309–18.
Nakajima, H., et al. “Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity.Eur J Immunol, vol. 30, no. 11, Nov. 2000, pp. 3309–18. Pubmed, doi:10.1002/1521-4141(200011)30:11<3309::AID-IMMU3309>3.0.CO;2-3.
Nakajima H, Cella M, Bouchon A, Grierson HL, Lewis J, Duckett CS, Cohen JI, Colonna M. Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity. Eur J Immunol. 2000 Nov;30(11):3309–3318.
Journal cover image

Published In

Eur J Immunol

DOI

ISSN

0014-2980

Publication Date

November 2000

Volume

30

Issue

11

Start / End Page

3309 / 3318

Location

Germany

Related Subject Headings

  • Signaling Lymphocytic Activation Molecule Family
  • Signaling Lymphocytic Activation Molecule Associated Protein
  • Signal Transduction
  • Receptors, Immunologic
  • Membrane Glycoproteins
  • Lymphoproliferative Disorders
  • Killer Cells, Natural
  • Intracellular Signaling Peptides and Proteins
  • Immunology
  • Humans