Kindlin-3–independent adhesion of neutrophils from patients with leukocyte adhesion deficiency type III

Activation of hematopoietic integrins to a high-affinity ligand-binding conformation requires interaction with several cytoplasmic proteins, including kindlin-3.2 The importance of kindlin-3 is illustrated by leukocyte adhesion deficiency type III (LAD-III), a rare autosomal-recessive syndrome cause...

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Veröffentlicht in:	Journal of allergy and clinical immunology Jg. 133; H. 4; S. 1215 - 1218.e3
Hauptverfasser:	van de Vijver, Edith, Tool, Anton T.J., Sanal, Özden, Çetin, Mualla, Ünal, Sule, Aytac, Selin, Seeger, Karl, Pagliara, Daria, Rutella, Sergio, van den Berg, Timo K., Kuijpers, Taco W.
Format:	Journal Article
Sprache:	Englisch
Veröffentlicht:	New York, NY Elsevier Inc 01.04.2014 Elsevier Elsevier Limited
Schlagworte:	Allergy and Immunology Biological and medical sciences Cell Adhesion - drug effects Cell Adhesion - genetics Cell Adhesion - immunology Cloning Fundamental and applied biological sciences. Psychology Fundamental immunology Hematologic and hematopoietic diseases Humans Immunodeficiencies Immunodeficiencies. Immunoglobulinopathies Immunopathology Kinases Leukocyte-Adhesion Deficiency Syndrome - diagnosis Leukocyte-Adhesion Deficiency Syndrome - genetics Leukocyte-Adhesion Deficiency Syndrome - metabolism Ligands Medical sciences Membrane Proteins - genetics Membrane Proteins - metabolism Mutation Neoplasm Proteins - genetics Neoplasm Proteins - metabolism Neutrophils - drug effects Neutrophils - immunology Neutrophils - metabolism Other diseases. Hematologic involvement in other diseases Patients Phosphates Proteins Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Human Immunopathology Immunology Hemopathy Neutrophil Immune deficiency Adhesion Leukocyte adhesion deficiency Leukocyte disease Genetic disease
ISSN:	0091-6749, 1097-6825, 1097-6825
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Zusammenfassung:	Activation of hematopoietic integrins to a high-affinity ligand-binding conformation requires interaction with several cytoplasmic proteins, including kindlin-3.2 The importance of kindlin-3 is illustrated by leukocyte adhesion deficiency type III (LAD-III), a rare autosomal-recessive syndrome caused by mutations in FERMT3, the gene encoding kindlin-3.3 LAD-III manifests with recurrent nonpurulent bacterial and fungal infections as well as with a Glanzmann thrombasthenia-like bleeding tendency, caused by normal expression but defective activation of integrins on leukocytes and platelets.4 Although the relevance of kindlin-3 is no longer disputed, it is still unclear how and when it contributes to the activation of the Î²2 integrins. Adhesion of LAD-III neutrophils was strongly decreased when induced with Toll-like receptor (TLR) ligands Pam3Cys and lipopolysaccharide-binding protein/lipopolysaccharide or with G-protein-coupled receptor (GPCR) ligands platelet-activation factor and C5a.
Bibliographie:	ObjectType-Case Study-3 SourceType-Scholarly Journals-1 ObjectType-Correspondence-1 content type line 14 ObjectType-Report-2 ObjectType-Case Study-2 ObjectType-Correspondence-3 content type line 23 ObjectType-Article-4 ObjectType-Report-1
ISSN:	0091-6749 1097-6825 1097-6825
DOI:	10.1016/j.jaci.2013.10.020