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Journal of Clinical Immunology

, Volume 34, Issue 8, pp 900–903 | Cite as

Leukocyte Adhesion Deficiency III - When Integrins Activation Fails

  • Amos Etzioni
Key Review Article

Leukocyte adhesion deficiency (LAD) syndromes are a group of genetic conditions in which adhesion of leukocyte to the endothelium is defective in one of the various phases of the adhesion cascade [1]. The first described defect, LAD I, was described more than 30 years ago, is due to mutation in the gene encoded for the β2 subunit of the integrin which is essential for the firm adhesion of leukocytes to the blood vessel endothelium [2]. In LAD II the first phase of the adhesion cascade, the rolling phase is defective. In this condition the ligand for the selectin on the leukocyte is absence due to a defect in fucosylation [3].

Still, most of the attention in the field is focused today on the third syndrome, LAD III, through which many new aspects regarding leukocyte adhesion were discovered.

In 1997 Kuijpers et al. [4] described a Turkish child of a consanguineous parents who presented with a mild LAD I phenotype, mainly recurrent bacterial infections without apparent pus formation and...

Keywords

Leukocyte Adhesion Deficiency III kindlin 3 immunodeficiency 

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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Ruth Children Hospital, HaifaRappaport Medical SchoolTechnionIsrael
  2. 2.Ruth Children HospitalRambam Medical CampusHaifaIsrael

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