| pubmed-article:20307660 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:20307660 | lifeskim:mentions | umls-concept:C0439660 | lld:lifeskim |
| pubmed-article:20307660 | lifeskim:mentions | umls-concept:C0009333 | lld:lifeskim |
| pubmed-article:20307660 | lifeskim:mentions | umls-concept:C0009326 | lld:lifeskim |
| pubmed-article:20307660 | lifeskim:mentions | umls-concept:C0151650 | lld:lifeskim |
| pubmed-article:20307660 | lifeskim:mentions | umls-concept:C1333206 | lld:lifeskim |
| pubmed-article:20307660 | lifeskim:mentions | umls-concept:C0205421 | lld:lifeskim |
| pubmed-article:20307660 | lifeskim:mentions | umls-concept:C1517945 | lld:lifeskim |
| pubmed-article:20307660 | pubmed:issue | 5 | lld:pubmed |
| pubmed-article:20307660 | pubmed:dateCreated | 2010-7-12 | lld:pubmed |
| pubmed-article:20307660 | pubmed:abstractText | Alport syndrome is a hereditary type IV collagen disease leading to progressive renal fibrosis, hearing loss and ocular changes. End stage renal failure usually develops during adolescence. COL4A3-/- mice serve as an animal model for progressive renal scarring in Alport syndrome. The present study evaluates the role of Discoidin Domain Receptor 1 (DDR1) in cell-matrix interaction involved in pathogenesis of Alport syndrome including renal inflammation and fibrosis. DDR1/COL4A3 Double-knockouts were compared to COL4A3-/- mice with 50% or 100% expression of DDR1, wildtype controls and to DDR1-/- COL4A3+/+ controls for over 6years. Double-knockouts lived 47% longer, mice with 50% DDR1 lived 29% longer and showed improved renal function (reduction in proteinuria and blood urea nitrogen) compared to animals with 100% DDR1 expression. Loss of DDR1 reduced proinflammatory, profibrotic cells via signaling of TGFbeta, CTGF, NFkappaB and IL-6 and decreased deposition of extracellular matrix. Immunogold-staining and in-situ hybridisation identified podocytes as major players in DDR1-mediated fibrosis and inflammation within the kidney. In summary, glomerular epithelial cells (podocytes) express DDR1. Loss of DDR1-expression in the kidney delayed renal fibrosis and inflammation in hereditary type IV collagen disease. This supports our hypothesis that podocyte-matrix interaction via collagen receptors plays an important part in progression of renal fibrosis in Alport disease. The blockade of collagen-receptor DDR1 might serve as an important new therapeutic concept in progressive fibrotic and inflammatory diseases in the future. | lld:pubmed |
| pubmed-article:20307660 | pubmed:language | eng | lld:pubmed |
| pubmed-article:20307660 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20307660 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:20307660 | pubmed:month | Jun | lld:pubmed |
| pubmed-article:20307660 | pubmed:issn | 1569-1802 | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:KretzlerMatth... | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:SegererStepha... | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:MiosgeNicolai... | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:VogelWolfgang... | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:GrossOliverO | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:WeberManfredM | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:KangHee... | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:GirgertRainer... | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:BeirowskiBogd... | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:MüllerGerhard... | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:BusseAnn-Chri... | lld:pubmed |
| pubmed-article:20307660 | pubmed:author | pubmed-author:KruegelJennyJ | lld:pubmed |
| pubmed-article:20307660 | pubmed:copyrightInfo | Copyright (c) 2010 Elsevier B.V. All rights reserved. | lld:pubmed |
| pubmed-article:20307660 | pubmed:issnType | Electronic | lld:pubmed |
| pubmed-article:20307660 | pubmed:volume | 29 | lld:pubmed |
| pubmed-article:20307660 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:20307660 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:20307660 | pubmed:pagination | 346-56 | lld:pubmed |
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| pubmed-article:20307660 | pubmed:year | 2010 | lld:pubmed |
| pubmed-article:20307660 | pubmed:articleTitle | Loss of collagen-receptor DDR1 delays renal fibrosis in hereditary type IV collagen disease. | lld:pubmed |
| pubmed-article:20307660 | pubmed:affiliation | Department of Nephrology & Rheumatology, University Medicine Goettingen Robert-Koch Strasse 40, Goettingen, Germany. gross.oliver@med.uni-goettingen.de | lld:pubmed |
| pubmed-article:20307660 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:20307660 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| entrez-gene:12305 | entrezgene:pubmed | pubmed-article:20307660 | lld:entrezgene |
| entrez-gene:12828 | entrezgene:pubmed | pubmed-article:20307660 | lld:entrezgene |
| http://linkedlifedata.com/r... | entrezgene:pubmed | pubmed-article:20307660 | lld:entrezgene |
