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.

The lack of BTK does not impair monocytes and polymorphonuclear cells functions in X-linked agammaglobulinemia under treatment with intravenous immunoglobulin replacement / Cavaliere, Filomena Monica; Prezzo, Alessandro; Bilotta, Caterina; Iacobini, Metello; Quinti, Isabella. - In: PLOS ONE. - ISSN 1932-6203. - ELETTRONICO. - 12:4(2017), p. e0175961. [10.1371/journal.pone.0175961]

The lack of BTK does not impair monocytes and polymorphonuclear cells functions in X-linked agammaglobulinemia under treatment with intravenous immunoglobulin replacement

CAVALIERE, Filomena Monica;PREZZO, ALESSANDRO;IACOBINI, Metello;QUINTI, Isabella
2017

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.
2017
Adult; Agammaglobulinemia; Calcium; Calcium Chelating Agents; Case-Control Studies; Cell Movement; Drug Administration Schedule; Gene Expression Regulation; Genetic Diseases, X-Linked; Humans; Immunoglobulins, Intravenous; Leukocytes, Mononuclear; Male; Middle Aged; Monocytes; Phagocytosis; Phenotype; Protein-Tyrosine Kinases; Reactive Oxygen Species; Receptors, IgG; Respiratory Burst; Signal Transduction; Medicine (all); Biochemistry, Genetics and Molecular Biology (all); Agricultural and Biological Sciences (all)
01 Pubblicazione su rivista::01a Articolo in rivista
The lack of BTK does not impair monocytes and polymorphonuclear cells functions in X-linked agammaglobulinemia under treatment with intravenous immunoglobulin replacement / Cavaliere, Filomena Monica; Prezzo, Alessandro; Bilotta, Caterina; Iacobini, Metello; Quinti, Isabella. - In: PLOS ONE. - ISSN 1932-6203. - ELETTRONICO. - 12:4(2017), p. e0175961. [10.1371/journal.pone.0175961]
File allegati a questo prodotto
File Dimensione Formato  
Cavaliere_Lack-BTK_2016.pdf

accesso aperto

Note: Articolo principale
Tipologia: Documento in Post-print (versione successiva alla peer review e accettata per la pubblicazione)
Licenza: Tutti i diritti riservati (All rights reserved)
Dimensione 3.52 MB
Formato Adobe PDF
3.52 MB Adobe PDF

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/957554
Citazioni
  • ???jsp.display-item.citation.pmc??? 10
  • Scopus 20
  • ???jsp.display-item.citation.isi??? 17
social impact