title: Toll-like receptors, immunoproteasome and regulatory T cells in children with Henoch-Schönlein purpura and primary IgA nephropathy.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Nephrology, Dialysis and Transplantation, University Hospital Città della Salute e della Scienza di Torino, Regina Margherita Hospital, Piazza Polonia 94, 1016, Turin, Italy.
authors.forename: Maria Elena
authors.initials: ME
authors.lastname: Donadio
authors.forename: Elisa
authors.initials: E
authors.lastname: Loiacono
authors.forename: Licia
authors.initials: L
authors.lastname: Peruzzi
authors.forename: Alessandro
authors.initials: A
authors.lastname: Amore
authors.forename: Roberta
authors.initials: R
authors.lastname: Camilla
authors.forename: Federica
authors.initials: F
authors.lastname: Chiale
authors.forename: Luca
authors.initials: L
authors.lastname: Vergano
authors.forename: Alberto
authors.initials: A
authors.lastname: Boido
authors.forename: Margherita
authors.initials: M
authors.lastname: Conrieri
authors.forename: Manuela
authors.initials: M
authors.lastname: Bianciotto
authors.forename: Francesca Maria
authors.initials: FM
authors.lastname: Bosetti
authors.forename: Rosanna
authors.initials: R
authors.lastname: Coppo
mesh_terms: D002648:Child; D005260:Female; D005922:Glomerulonephritis, IGA / Q000276:immunology*; D006801:Humans; D011695:IgA Vasculitis / Q000276:immunology*; D008297:Male; D046988:Proteasome Endopeptidase Complex / Q000276:immunology*; D060888:Real-Time Polymerase Chain Reaction; D050378:T-Lymphocytes, Regulatory / Q000276:immunology*; D051193:Toll-Like Receptors / Q000276:immunology*
chemical_list: D051193:Toll-Like Receptors; D046988:Proteasome Endopeptidase Complex