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Published in: Pediatric Nephrology 9/2014

01-09-2014 | Original Article

Toll-like receptors, immunoproteasome and regulatory T cells in children with Henoch–Schönlein purpura and primary IgA nephropathy

Authors: Maria Elena Donadio, Elisa Loiacono, Licia Peruzzi, Alessandro Amore, Roberta Camilla, Federica Chiale, Luca Vergano, Alberto Boido, Margherita Conrieri, Manuela Bianciotto, Francesca Maria Bosetti, Rosanna Coppo

Published in: Pediatric Nephrology | Issue 9/2014

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Abstract

Background

Henoch–Schönlein purpura (HSP) nephritis and primary IgA nephropathy (pIgAN) present with glomerular IgA deposits, but differ with regard to clinical features. The suspected involvement of different immune system pathways is largely unknown.

Methods

This study was aimed at investigating some of the immunological features including Toll-like receptors (TLR), proteasome (PS)/immunoproteasome (iPS) switch, and the regulatory T cell system (Treg/Th17 cells) in 63 children with HSP with/without renal involvement and in 25 with pIgAN. Real-time PRC (Taqman) was used to quantify mRNA levels in peripheral blood mononuclear cells (PBMC).

Results

The expression of mRNAs encoding for TLR4 in both HSP and pIgAN was higher than in controls (HC) and in both diseases FoxP3mRNA and TGF-β1mRNA expression was significantly lower than in HC. A switch from PS to iPS (LMP2/β1) was detected only in PBMC of HSP and it correlated with the level of TLR2mRNA, which was selectively increased only in children with HSP.

Conclusion

Children with HSP and pIgAN present with similar signs of engagement of the innate immunity and regulatory T cell depression. The increased immunoproteasome switch, which correlated with TLR2 activation, may suggest an innate immunity pathway peculiar to HSP vasculitic presentation. This research area also deserves further investigation for possible therapeutic applications.
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Metadata
Title
Toll-like receptors, immunoproteasome and regulatory T cells in children with Henoch–Schönlein purpura and primary IgA nephropathy
Authors
Maria Elena Donadio
Elisa Loiacono
Licia Peruzzi
Alessandro Amore
Roberta Camilla
Federica Chiale
Luca Vergano
Alberto Boido
Margherita Conrieri
Manuela Bianciotto
Francesca Maria Bosetti
Rosanna Coppo
Publication date
01-09-2014
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 9/2014
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-014-2807-6

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