TAILIEUCHUNG - Báo cáo khoa học: Leukocyte adhesion deficiency II Advances and open questions

Leukocyte adhesion deficiency II (LAD II) belongs to a group of human congenital diseases in which the interactions of leukocytes with the vascular endothelium are strongly impaired. LAD II is based on a defect in the syn-thesis of fucosylated glycostructures. This leads to an immunodeficiency owing to the absence of functional selectin ligands and to strong psycho-motor defects, as a result of as-yet unknown reasons. | MINIREVIEW Leukocyte adhesion deficiency II Advances and open questions Sviatlana Yakubenia and Martin K. Wild Max Planck Institute for Molecular Biomedicine and Institute of Cell Biology ZMBE University of Munster Munster Germany Keywords fucosylation GDP-fucose transporter immunodeficiency leukocyte adhesion deficiency II Notch Correspondence M. K. Wild Max Planck Institute for Molecular Biomedicine Von-Esmarch-Strasse 56 48149 Munster Germany Fax 49 251 8352236 Tel 49 251 8352180 E-mail wildm@ Leukocyte adhesion deficiency II LAD II belongs to a group of human congenital diseases in which the interactions of leukocytes with the vascular endothelium are strongly impaired. LAD II is based on a defect in the synthesis of fucosylated glycostructures. This leads to an immunodeficiency owing to the absence of functional selectin ligands and to strong psychomotor defects as a result of as-yet unknown reasons. In this review we focused on the current controversies and open questions that have arisen from recent studies on the genetic defect therapy and the basis of psychomotor defects in LAD II. Received 15 May 2006 accepted 14 July 2006 doi In order to extravasate into inflamed tissues or to home to secondary lymphoid organs leukocytes have to interact with endothelial cells in a sequence of adhesive events termed the multistep adhesion cascade . The cascade includes selectin-mediated tethering and rolling chemokine-dependent leukocyte activation integrin-mediated firm adhesion and finally transmigration into tissues. There are three types of leukocyte adhesion deficiencies LAD I-III that block these interactions. LAD I is caused by the defective expression or function of the P2 integrin subunit 1-3 whereas in LAD III the activation of integrins is affected 4-6 . LAD II also known as congenital disorder of glycosylation IIc CDG IIc is a rare congenital disease which interrupts the adhesion cascade at the first step namely

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