Publications

Authors:

Giacomelli, Bm; Tamassia, Nicola; Moratto, D; Bertolini, P; Ricci, G; Bertulli, C; Plebani, A; Cassatella, Marco Antonio; Bazzoni, Flavia; Badolato, R.

Title:

SH2 domain mutations of STAT3 gene result in impairment of IL-10 function in hyper-IgE syndrome patients

Year:

2011

Type of item:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Language:

Inglese

Format:

A Stampa

Referee:

Sì

Name of journal:

European Journal of Immunology

ISSN of journal:

0014-2980

N° Volume:

41

Number or Folder:

10

Page numbers:

3075-3084

Keyword:

Interleukin-10; hyper-IgE syndrome; monocytes; neutrophils; dendritic cells; interleukin-1 receptor antagonist (IL-1ra)

Short description of contents:

Autosomal-dominant hyper-IgE syndrome (AD-HIES) is a primary immunodeficiency caused by STAT3 mutations. This inherited condition is characterized by eczema, staphylococcal cold abscesses, and recurrent pulmonary infections. Because STAT3 is involved in IL-10 signaling, we examined the immunoregulatory role of IL-10 in inflammation by studying the effects of IL-10 on monocytes, neutrophils, and monocyte-derived dendritic cells (DC) from HIES subjects. Analysis of gene expression in PBMCs and neutrophils stimulated with LPS in the presence of IL-10 in HIES patients showed reduced expression of IL1RN, which encodes interleukin-1 receptor antagonist (IL-1ra), and SOCS3 mRNA but increased CXCL8 mRNA expression. Moreover, secretion of the anti-inflammatory protein IL-1ra was reduced in AD-HIES patients. DCs from HIES patients secreted higher levels of TNF-á, IL-6, and, to a lesser extent, IL-12 when cells were cultured in the presence of IL-10. These results suggest that IL-10 activity is affected in myeloid cells (e.g., monocytes, DCs) of HIES patients. Impairment of IL-10 signaling in patients with autosomal-dominant HIES might result in an altered balance between pro-inflammatory and anti-inflammatory signals and might lead to persistent inflammation and delayed healing after infections.

Web page:

http://onlinelibrary.wiley.com/doi/10.1002/eji.201141721/pdf

Product ID:

61288

Handle IRIS:

11562/359857

Deposited On:

September 30, 2011

Last Modified:

November 15, 2022

Bibliographic citation:

Giacomelli, Bm; Tamassia, Nicola; Moratto, D; Bertolini, P; Ricci, G; Bertulli, C; Plebani, A; Cassatella, Marco Antonio; Bazzoni, Flavia; Badolato, R., SH2 domain mutations of STAT3 gene result in impairment of IL-10 function in hyper-IgE syndrome patients «European Journal of Immunology» , vol. 41 , n. 10 , 2011 , pp. 3075-3084

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