Cargando…
Regulating complement in the kidney: insights from CFHR5 nephropathy
Complement factor H related protein 5 (CFHR5) nephropathy is a monogenic disorder of complement regulation that is endemic in Cyprus. The disease is characterised by haematuria, C3 glomerulonephritis and kidney failure. Its identification suggests a role for the CFHR5 protein in the regulation of co...
Autores principales: | , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Company of Biologists Limited
2011
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3209641/ https://www.ncbi.nlm.nih.gov/pubmed/22065842 http://dx.doi.org/10.1242/dmm.008052 |
_version_ | 1782215684798808064 |
---|---|
author | Gale, Daniel P. Pickering, Matthew C. |
author_facet | Gale, Daniel P. Pickering, Matthew C. |
author_sort | Gale, Daniel P. |
collection | PubMed |
description | Complement factor H related protein 5 (CFHR5) nephropathy is a monogenic disorder of complement regulation that is endemic in Cyprus. The disease is characterised by haematuria, C3 glomerulonephritis and kidney failure. Its identification suggests a role for the CFHR5 protein in the regulation of complement in the kidney. In this review, we discuss how studying CFHR5 nephropathy can contribute to our understanding of the role of complement in kidney diseases such as dense deposit disease, C3 glomerulonephritis and atypical haemolytic uraemic syndrome. |
format | Online Article Text |
id | pubmed-3209641 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | The Company of Biologists Limited |
record_format | MEDLINE/PubMed |
spelling | pubmed-32096412011-11-10 Regulating complement in the kidney: insights from CFHR5 nephropathy Gale, Daniel P. Pickering, Matthew C. Dis Model Mech Clinical Puzzle Complement factor H related protein 5 (CFHR5) nephropathy is a monogenic disorder of complement regulation that is endemic in Cyprus. The disease is characterised by haematuria, C3 glomerulonephritis and kidney failure. Its identification suggests a role for the CFHR5 protein in the regulation of complement in the kidney. In this review, we discuss how studying CFHR5 nephropathy can contribute to our understanding of the role of complement in kidney diseases such as dense deposit disease, C3 glomerulonephritis and atypical haemolytic uraemic syndrome. The Company of Biologists Limited 2011-11 /pmc/articles/PMC3209641/ /pubmed/22065842 http://dx.doi.org/10.1242/dmm.008052 Text en © 2011. Published by The Company of Biologists Ltd This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial Share Alike License (http://creativecommons.org/licenses/by-nc-sa/3.0), which permits unrestricted non-commercial use, distribution and reproduction in any medium provided that the original work is properly cited and all further distributions of the work or adaptation are subject to the same Creative Commons License terms. |
spellingShingle | Clinical Puzzle Gale, Daniel P. Pickering, Matthew C. Regulating complement in the kidney: insights from CFHR5 nephropathy |
title | Regulating complement in the kidney: insights from CFHR5 nephropathy |
title_full | Regulating complement in the kidney: insights from CFHR5 nephropathy |
title_fullStr | Regulating complement in the kidney: insights from CFHR5 nephropathy |
title_full_unstemmed | Regulating complement in the kidney: insights from CFHR5 nephropathy |
title_short | Regulating complement in the kidney: insights from CFHR5 nephropathy |
title_sort | regulating complement in the kidney: insights from cfhr5 nephropathy |
topic | Clinical Puzzle |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3209641/ https://www.ncbi.nlm.nih.gov/pubmed/22065842 http://dx.doi.org/10.1242/dmm.008052 |
work_keys_str_mv | AT galedanielp regulatingcomplementinthekidneyinsightsfromcfhr5nephropathy AT pickeringmatthewc regulatingcomplementinthekidneyinsightsfromcfhr5nephropathy |