Glycoprotein-180 deficiency: genetics and abnormal neutrophil activation
| Autor: | SJ Weisman, RL Berkow, G Plautz, M Torres, WA McGuire, TD Coates, RA Haak, A Floyd, R Jersild, RL Baehner |
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| Rok vydání: | 1985 |
| Předmět: |
Blood Protein Disorders
Membrane Glycoproteins Rosette Formation Leukocytosis Membrane Fluidity Neutrophils Chemotaxis Immunology hemic and immune systems Cell Biology Hematology Receptors Fc Biochemistry Membrane Potentials Receptors Complement Skin Window Technique Phagocytosis Superoxides Child Preschool Cell Migration Inhibition Cell Adhesion Humans Female Glycoproteins |
| Zdroj: | Blood. 65(3) |
| ISSN: | 0006-4971 |
| Popis: | Neutrophil function was studied in a patient with polymorphonuclear leukocyte (PMN) glycoprotein-180 deficiency and in her parents. PMNs of the patient had abnormal chemotaxis, phagocytosis, adherence, surface charge, and membrane-associated events of activation. Selective defects to C3b, immunoglobulin G (IgG), phorbol myristate acetate (PMA) and N- formyl-methionyl-leucyl-phenylalanine (FMLP) are described, although C3b receptor density was normal. The parents were found to have abnormal adherence to nylon-wool fibers, abnormal transmembrane potential depolarization with PMA, and reduced amounts of glycoprotein- 180 in their PMNs. These studies provide further evidence that the oxidative burst has several different pathways for activation. They demonstrate that the absence of a single PMN surface glycoprotein is associated with a broad spectrum of PMN functional abnormalities. Finally, the observations made in the parents support an autosomal recessive mode of inheritance. |
| Databáze: | OpenAIRE |
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