Source:http://linkedlifedata.com/resource/pubmed/id/21252520
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rdf:type | |
lifeskim:mentions | |
pubmed:dateCreated |
2011-1-21
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pubmed:abstractText |
Membranoproliferative glomerulonephritis (MPGN) is characterised by mesangial expansion and hypercellularity and capillary wall thickening with capillary wall and mesangial deposits of immunoglobulin and/or complement. Two main forms are described in humans: MPGN type I with subendothelial and mesangial electron-dense deposits on electron microscopy, and MPGN type II, or dense deposit disease, with electron dense transformation of the glomerular capillary wall. Spontaneous MPGN type I has been described in dogs and sheep in association with C3 deficiency. Induced models of MPGN type I have been described in mice with cryoglobulinaemia. Glomerulonephritis resembling MPGN type II has occurred spontaneously in pigs that have a genetic deficiency of the complement control protein factor H. The animals develop capillary wall deposits of C3 before birth. Mice have been genetically engineered with a deficiency of factor H and similarly develop glomerular capillary wall C3 with MPGN. This model has been used to study both pathogenesis and therapeutic interventions. In particular, MPGN associated with factor H deficiency is absolutely dependent on both the ability to activate C3 and on the ability of factor I to cleave C3b. There is an important role for C5 activation in the development of glomerular inflammation in this model. Factor H dysfunction is associated with an increased susceptibility to complement-activating nephrotoxic insults and in these scenarios C5 activation appears to play a major role in mediating glomerular injury.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:issn |
1662-2782
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pubmed:author | |
pubmed:copyrightInfo |
Copyright © 2011 S. Karger AG, Basel.
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pubmed:issnType |
Electronic
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pubmed:volume |
169
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
198-210
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pubmed:meshHeading |
pubmed-meshheading:21252520-Animals,
pubmed-meshheading:21252520-Complement C3,
pubmed-meshheading:21252520-Complement C5,
pubmed-meshheading:21252520-Complement Factor H,
pubmed-meshheading:21252520-Disease Models, Animal,
pubmed-meshheading:21252520-Dogs,
pubmed-meshheading:21252520-Glomerulonephritis, Membranoproliferative,
pubmed-meshheading:21252520-Humans,
pubmed-meshheading:21252520-Mice,
pubmed-meshheading:21252520-Swine
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pubmed:year |
2011
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pubmed:articleTitle |
Experimental models of membranoproliferative glomerulonephritis, including dense deposit disease.
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pubmed:affiliation |
Centre for Complement and Inflammation Research, Department of Medicine, Imperial College, London, UK.
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pubmed:publicationType |
Journal Article
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