Abberant Cytosolic Calcium Ion Mobilization in Chronic Granulomatous Disease Neutrophils
| Autor: | Anders Åhlin, Mikael Heimbürger, Jan Palmblad, Anders Hansson |
|---|---|
| Rok vydání: | 2004 |
| Předmět: |
Membrane potential
congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Mutation Chemistry Leukotriene B4 Immunology chemistry.chemical_element hemic and immune systems Chemotaxis Phosphatidic acid Phospholipase Calcium medicine.disease_cause medicine.disease chemistry.chemical_compound Endocrinology Chronic granulomatous disease hemic and lymphatic diseases Internal medicine medicine Immunology and Allergy |
| Zdroj: | Inflammation. 28:133-138 |
| ISSN: | 0360-3997 |
| DOI: | 10.1023/b:ifla.0000039559.96659.d9 |
| Popis: | Beside the inability to produce superoxide ions, neutrophils (PMN) from chronic granulomatous disease (CGD) patients show other functional defects, e.g. abnormal membrane potential reactions. We observed that PMN from a female CGD carrier, with a discrete mutation in one allele of the pg91(phox) gene and exhibiting extreme lyonization, showed a consistently and remarkably delayed PMN cytosolic calcium response to the tripeptide fMLP or leukotriene B4 (LTB4). In keeping with results from other CGD patients, membrane potential changes were abnormal, whereas chemotaxis and adherence was normal. Since phospholipase D-generated metabolites are important for calcium transients we examined the generation of phosphatidic acid, but found that to be normal. A male CGD patient with pg91(phox) deficiency exhibited a trend toward prolongation of this calcium response, whereas two other CGD patients (one with p47 and one with 67(phox) deficiencies) had normal calcium transients. Thus, our finding points to a defect of the stimulus response coupling for fMLP and LTB4, which is supposed to be characteristic for this patient or a subset of CGD patients. |
| Databáze: | OpenAIRE |
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