| Autor: |
Weisman, SJ, Berkow, RL, Plautz, G, Torres, M, McGuire, WA, Coates, TD, Haak, RA, Floyd, A, Jersild, R, Baehner, RL |
| Zdroj: |
Blood; March 1985, Vol. 65 Issue: 3 p696-704, 9p |
| Abstrakt: |
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: |
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| Externí odkaz: |
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