Abstract |
The lack of BTK in X-linked agammaglobulinemia (XLA) patients does not affect monocytes and polymorphonuclear cells (PMN) phenotype and functions. In this study, we show that XLA patients had an increased frequency of the intermediate monocytes subset and that BTK-deficient monocytes and PMN had a normal expression of receptors involved in the activation and cellular responses. We demonstrate that BTK is not required for migration, phagocytosis and the production of reactive oxygen species (ROS) following engagement of FC gamma receptors (FcγR). XLA monocytes and PMN showed an efficient calcium (Ca2+)-independent activation of oxidative burst, suggesting that oxidative burst is less dependent by Ca2+ mobilization. The phagocytosis was functional and it remained unaltered also after Ca2+ chelation, confirming the independence of phagocytosis on Ca2+ mobilization. Intravenous immunoglobulin ( IVIg) infusion exerted an anti-inflammatory effect by reducing the frequency of pro-inflammatory monocytes. In monocytes, the IVIg reduce the oxidative burst and phagocytosis even if these functions remained efficient.
|
Authors | Filomena Monica Cavaliere, Alessandro Prezzo, Caterina Bilotta, Metello Iacobini, Isabella Quinti |
Journal | PloS one
(PLoS One)
Vol. 12
Issue 4
Pg. e0175961
( 2017)
ISSN: 1932-6203 [Electronic] United States |
PMID | 28422989
(Publication Type: Journal Article)
|
Chemical References |
- Calcium Chelating Agents
- Immunoglobulins, Intravenous
- Reactive Oxygen Species
- Receptors, IgG
- Protein-Tyrosine Kinases
- Agammaglobulinaemia Tyrosine Kinase
- BTK protein, human
- Calcium
|
Topics |
- Adult
- Agammaglobulinaemia Tyrosine Kinase
- Agammaglobulinemia
(drug therapy, genetics, immunology, pathology)
- Calcium
(metabolism)
- Calcium Chelating Agents
(pharmacology)
- Case-Control Studies
- Cell Movement
(drug effects)
- Drug Administration Schedule
- Gene Expression Regulation
- Genetic Diseases, X-Linked
(drug therapy, genetics, immunology, pathology)
- Humans
- Immunoglobulins, Intravenous
(therapeutic use)
- Leukocytes, Mononuclear
(drug effects, immunology, pathology)
- Male
- Middle Aged
- Monocytes
(drug effects, immunology, pathology)
- Phagocytosis
(drug effects)
- Phenotype
- Protein-Tyrosine Kinases
(deficiency, genetics, immunology)
- Reactive Oxygen Species
(immunology, metabolism)
- Receptors, IgG
(genetics, immunology)
- Respiratory Burst
(drug effects, immunology)
- Signal Transduction
|