Leucocyte adhesion protein deficiency in Irish setter dogs

Investigation of 12 Irish setter puppies from six litters with severe recurrent infections, neutrophilia and low body weight revealed a leucocyte adhesion protein deficiency with a total lack of CD11b and CD18. Their neutrophil function was severely impaired with a totally absent capacity to ingest...

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Veröffentlicht in:Veterinary immunology and immunopathology 1992-05, Vol.32 (3), p.261-280
Hauptverfasser: Trowald-Wigh, Gunilla, Håkansson, Lena, Johannisson, Anders, Norrgren, Leif, Hård af Segerstad, Carl
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container_end_page 280
container_issue 3
container_start_page 261
container_title Veterinary immunology and immunopathology
container_volume 32
creator Trowald-Wigh, Gunilla
Håkansson, Lena
Johannisson, Anders
Norrgren, Leif
Hård af Segerstad, Carl
description Investigation of 12 Irish setter puppies from six litters with severe recurrent infections, neutrophilia and low body weight revealed a leucocyte adhesion protein deficiency with a total lack of CD11b and CD18. Their neutrophil function was severely impaired with a totally absent capacity to ingest C3b-opsonized particles, a significantly impaired capacity to ingest IgG-opsonized particles and significantly diminished adherence to nylon wool when compared with neutrophils from healthy control dogs. The chemiluminescence of patient neutrophils activated by C3b-opsonized particles was, consequently, significantly decreased compared with that of control neutrophils, while the respiratory burst assayed by phorbolmyristate acid (PMA) stimulated nitroblue tetrazolium (NBT)-reduction was normal in the patient group. Random migration and chemotactic responses of patient and control neutrophils, were similar. The etiology, pathogenesis and clinical manifestations of the Irish setter leucocyte adhesion deficiency were similar to that of the leucocyte adhesion deficiency in humans.
doi_str_mv 10.1016/0165-2427(92)90050-Z
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Their neutrophil function was severely impaired with a totally absent capacity to ingest C3b-opsonized particles, a significantly impaired capacity to ingest IgG-opsonized particles and significantly diminished adherence to nylon wool when compared with neutrophils from healthy control dogs. The chemiluminescence of patient neutrophils activated by C3b-opsonized particles was, consequently, significantly decreased compared with that of control neutrophils, while the respiratory burst assayed by phorbolmyristate acid (PMA) stimulated nitroblue tetrazolium (NBT)-reduction was normal in the patient group. Random migration and chemotactic responses of patient and control neutrophils, were similar. 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Animal models</topic><topic>Female</topic><topic>Flow Cytometry</topic><topic>Immunologic Deficiency Syndromes - blood</topic><topic>Immunologic Deficiency Syndromes - genetics</topic><topic>Immunologic Deficiency Syndromes - veterinary</topic><topic>Immunopathology</topic><topic>Luminescent Measurements</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Microscopy, Electron, Scanning</topic><topic>Neutrophils - immunology</topic><topic>Neutrophils - ultrastructure</topic><topic>Nitroblue Tetrazolium</topic><topic>Phagocytosis</topic><topic>Receptors, Leukocyte-Adhesion - genetics</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Trowald-Wigh, Gunilla</creatorcontrib><creatorcontrib>Håkansson, Lena</creatorcontrib><creatorcontrib>Johannisson, Anders</creatorcontrib><creatorcontrib>Norrgren, Leif</creatorcontrib><creatorcontrib>Hård af Segerstad, Carl</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Veterinary immunology and immunopathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Trowald-Wigh, Gunilla</au><au>Håkansson, Lena</au><au>Johannisson, Anders</au><au>Norrgren, Leif</au><au>Hård af Segerstad, Carl</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Leucocyte adhesion protein deficiency in Irish setter dogs</atitle><jtitle>Veterinary immunology and immunopathology</jtitle><addtitle>Vet Immunol Immunopathol</addtitle><date>1992-05</date><risdate>1992</risdate><volume>32</volume><issue>3</issue><spage>261</spage><epage>280</epage><pages>261-280</pages><issn>0165-2427</issn><eissn>1873-2534</eissn><abstract>Investigation of 12 Irish setter puppies from six litters with severe recurrent infections, neutrophilia and low body weight revealed a leucocyte adhesion protein deficiency with a total lack of CD11b and CD18. Their neutrophil function was severely impaired with a totally absent capacity to ingest C3b-opsonized particles, a significantly impaired capacity to ingest IgG-opsonized particles and significantly diminished adherence to nylon wool when compared with neutrophils from healthy control dogs. The chemiluminescence of patient neutrophils activated by C3b-opsonized particles was, consequently, significantly decreased compared with that of control neutrophils, while the respiratory burst assayed by phorbolmyristate acid (PMA) stimulated nitroblue tetrazolium (NBT)-reduction was normal in the patient group. Random migration and chemotactic responses of patient and control neutrophils, were similar. The etiology, pathogenesis and clinical manifestations of the Irish setter leucocyte adhesion deficiency were similar to that of the leucocyte adhesion deficiency in humans.</abstract><cop>Amsterdam</cop><pub>Elsevier B.V</pub><pmid>1352926</pmid><doi>10.1016/0165-2427(92)90050-Z</doi><tpages>20</tpages></addata></record>
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subjects Animals
Antigens, CD - genetics
Biological and medical sciences
Bone Marrow - immunology
CD11 Antigens
CD18 Antigens
Cell Adhesion
Chemotaxis
Dog Diseases - immunology
Dogs
Experimental and animal immunopathology. Animal models
Female
Flow Cytometry
Immunologic Deficiency Syndromes - blood
Immunologic Deficiency Syndromes - genetics
Immunologic Deficiency Syndromes - veterinary
Immunopathology
Luminescent Measurements
Male
Medical sciences
Microscopy, Electron, Scanning
Neutrophils - immunology
Neutrophils - ultrastructure
Nitroblue Tetrazolium
Phagocytosis
Receptors, Leukocyte-Adhesion - genetics
title Leucocyte adhesion protein deficiency in Irish setter dogs
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