Leukocyte adhesion deficiencies.

Abstract:

:Leukocyte trafficking from the blood stream to tissues is essential for continuous surveillance of foreign antigens. This dynamic process, designated as the leukocyte adhesion cascade, involves distinct steps. In leukocyte adhesion deficiency (LAD) I the firm adhesion of leukocyte to the endothelium is defective, due to mutations in the beta 2 integrin gene. LAD II is caused by mutations in the fucose transporter specific to the Golgi apparatus, leading to the absence of Sialyl Lewis X-the fucosylated ligand for the selectins-thus affecting the rolling phase, the first phase of the cascade. In LAD III, a primary activation defect occurs in beta integrins 1, 2, and 3. Recently, the genetic basis for LAD III has been revealed to involve mutations in kindlin-3, a newly recognized essential component of integrin activation-the second phase of the adhesion cascade. Until now, no human or animal models of defect in transmigration-the fourth and last phase of the cascade-has been described.

journal_name

Ann N Y Acad Sci

authors

Hanna S,Etzioni A

doi

10.1111/j.1749-6632.2011.06389.x

subject

Has Abstract

pub_date

2012-02-01 00:00:00

pages

50-5

eissn

0077-8923

issn

1749-6632

journal_volume

1250

pub_type

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