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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Vydané v:	Journal of allergy and clinical immunology Ročník 133; číslo 4; s. 1215 - 1218.e3
Hlavní autori:	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.
Médium:	Journal Article
Jazyk:	English
Vydavateľské údaje:	New York, NY Elsevier Inc 01.04.2014 Elsevier Elsevier Limited
Predmet:	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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Abstract	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.
AbstractList	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.
Author	Çetin, Mualla Sanal, Özden van den Berg, Timo K. Pagliara, Daria Aytac, Selin Kuijpers, Taco W. Ünal, Sule Seeger, Karl Tool, Anton T.J. van de Vijver, Edith Rutella, Sergio
Author_xml	– sequence: 1 givenname: Edith surname: van de Vijver fullname: van de Vijver, Edith organization: Emma Children’s Hospital, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands – sequence: 2 givenname: Anton T.J. surname: Tool fullname: Tool, Anton T.J. organization: Department of Blood Cell Research, Sanquin Research, and Landsteiner Laboratory, Amsterdam, The Netherlands – sequence: 3 givenname: Özden surname: Sanal fullname: Sanal, Özden organization: Pediatric Immunology Unit, Hacettepe University, Ankara, Turkey – sequence: 4 givenname: Mualla surname: Çetin fullname: Çetin, Mualla organization: Pediatric Hematology Unit, Hacettepe University, Ankara, Turkey – sequence: 5 givenname: Sule surname: Ünal fullname: Ünal, Sule organization: Pediatric Hematology Unit, Hacettepe University, Ankara, Turkey – sequence: 6 givenname: Selin surname: Aytac fullname: Aytac, Selin organization: Pediatric Hematology Unit, Hacettepe University, Ankara, Turkey – sequence: 7 givenname: Karl surname: Seeger fullname: Seeger, Karl organization: Department of Pediatric Oncology/Hematology, Otto-Heubner-Center for Pediatric and Adolescent Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany – sequence: 8 givenname: Daria surname: Pagliara fullname: Pagliara, Daria organization: Department of Pediatric Hematology/Oncology, IRCCS Bambino Gesù Children’s Hospital, Rome, Italy – sequence: 9 givenname: Sergio surname: Rutella fullname: Rutella, Sergio organization: Department of Pediatric Hematology/Oncology, IRCCS Bambino Gesù Children’s Hospital, Rome, Italy – sequence: 10 givenname: Timo K. surname: van den Berg fullname: van den Berg, Timo K. organization: Department of Blood Cell Research, Sanquin Research, and Landsteiner Laboratory, Amsterdam, The Netherlands – sequence: 11 givenname: Taco W. surname: Kuijpers fullname: Kuijpers, Taco W. email: t.w.kuijpers@amc.uva.nl organization: Emma Children’s Hospital, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
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SubjectTerms	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
Title	Kindlin-3–independent adhesion of neutrophils from patients with leukocyte adhesion deficiency type III
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