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NDT Advance Access originally published online on October 23, 2007
Nephrology Dialysis Transplantation 2008 23(2):462-465; doi:10.1093/ndt/gfm758
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


A mouse model of non-Shiga toxin-associated haemolytic uraemic syndrome*

Jessica Caprioli1 and Giuseppe Remuzzi1,2

1Mario Negri Institute for Pharmacological Research, Clinical Research Center for Rare Diseases, Aldo e Cele Daccò, Villa Camozzi, Ranica, Bergamo, Italy and 2Department of Nephrology and Dialysis, Azienda Ospedaliera, Ospedali Riuniti di Bergamo, Italy

Correspondence and offprint requests to: Jessica Caprioli, Transplant Research Center, Chiara Cucchi de Alessandri e Gilberto Crespi, Villa Camozzi, 3-24020 Ranica (BG), Italy. Tel: +39-035-4535355; Fax: +39-035-4535377; Email: caprioli@marionegri.it

Keywords: non-Stx-HUS; mouse model

The first 10% of the full text of this article appears below.



   Introduction
 
Impaired control of the complement system activation due to mutations in complement factor H (CFH) has been described in two apparently unrelated human diseases, membranoproliferative glomerulonephitis type II (MPGN2) and non-Shiga toxin-associated haemolytic uraemic syndrome (non-Stx-HUS). Mouse models of these diseases have been developed by Pickering et al., by knocking-out Cfh gene (MPGN2) and by subsequently transferring a mutated Cfh gene in the Cfh–/– background (non-Stx-HUS). The data obtained from the two models provided precious information to clarify the mechanisms that cause the disparate phenotypes underlying CFH genetic defect.



   MPGN2
 
MPGN2 is a rare cause of chronic nephritis characterized by the presence of dense deposits within the glomerular basement membrane (GBM), capillary wall thickening, mesangial cell proliferation and glomerular fibrosis [1,2].



   Non-Stx-HUS
 
Non-Shiga toxin-associated haemolytic uraemic syndrome (non-Stx-HUS) is a rare disease with manifestations of haemolytic anaemia, thrombocytopenia and renal failure. The clinical outcome is unfavourable, . . . [Full Text of this Article]



   CFH
 


   What did we learn from mouse models of MPGN2 and HUS?
 


   Clinical implications
 


   Take home message
 

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