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Leukocyte adhesion deficiency (LAD) II

A Etzioni1, L M Phillips, J C Paulson

  • 1Department of Pediatrics, Rambam Medical Center, B. Rappaport Medical School, Haifa, Israel.

Ciba Foundation Symposium
|January 1, 1995
PubMed
Summary

Two children with recurrent infections and neutrophil dysfunction were found to have a deficiency in sialyl Lewis X (SLe(x)), a key molecule for immune cell adhesion. This highlights SLe(x)

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Area of Science:

  • Immunology
  • Cell Biology
  • Genetics

Background:

  • Recurrent bacterial infections and neutrophil motility dysfunction in two children suggested an unknown adhesion deficiency.
  • The patients' rare Bombay blood group and Lewis negative status pointed towards a potential defect in fucose-containing carbohydrates, specifically sialyl Lewis X (SLe(x)).

Observation:

  • Neutrophils from the patients lacked detectable sialyl Lewis X (SLe(x)) expression.
  • Neutrophil adhesion to activated endothelial cells and binding to E- and P-selectin were significantly reduced.
  • In vivo studies showed markedly decreased neutrophil rolling and emigration to inflammation sites.

Findings:

  • Confirmed a deficiency in sialyl Lewis X (SLe(x)) as the cause of the observed neutrophil adhesion defect.
  • Demonstrated that the primary defect is a general fucose deficiency, impacting SLe(x) expression.
  • Established the critical role of endothelial selectins and their ligand, SLe(x), in neutrophil recruitment.

Implications:

  • Underscores the essential role of sialyl Lewis X (SLe(x)) and endothelial selectins in the innate immune response.
  • Provides insights into the mechanisms of neutrophil adhesion and migration.
  • Offers a potential target for therapeutic interventions in inflammatory and infectious diseases.

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