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Findings on Alport Syndrome Detailed by Investigators at University of Cologne (Collagen receptors integrin alpha2beta1 and discoidin domain receptor...

July 4, 2014



Findings on Alport Syndrome Detailed by Investigators at University of Cologne (Collagen receptors integrin alpha2beta1 and discoidin domain receptor 1 regulate maturation of the glomerular basement membrane and loss of integrin alpha2betal ...)

By a News Reporter-Staff News Editor at Health & Medicine Week -- Research findings on Alport syndrome are discussed in a new report. According to news originating from Cologne, Germany, by NewsRx correspondents, research stated, "Maturation of the glomerular basement membrane (GBM) is essential for maintaining the integrity of the renal filtration barrier. Impaired maturation causes proteinuria and renal fibrosis in the type IV collagen disease Alport syndrome."

Our news journalists obtained a quote from the research from the University of Cologne, "This study evaluates the role of collagen receptors in maturation of the GBM, matrix accumulation and renal fibrosis by using mice deficient for discoidin domain receptor 1 (DDR1), integrin subunit alpha 2 (ITGA2), and type IV collagen alpha 3 (COL4A3). Loss of both collagen receptors DDR1 and integrin alpha 2 beta 1 delays maturation of the GBM: due to a porous GBM filtration barrier high molecular weight proteinuria that more than doubles between day 60 and day 100. Thereafter, maturation of the GBM causes proteinuria to drop down to one tenth until day 200. Proteinuria and the porous GBM cause accumulation of glomerular and tubulointerstitial matrix, which both decrease significantly after GBM-maturation until day 250. In parallel, in a disease with impaired GBM-maturation such as Alport syndrome, loss of integrin alpha 2 beta 1 positively delays renal fibrosis: COL4A3(-/-) ITGA2(-/-) double knockouts exhibited reduced proteinuria and urea nitrogen compared to COL4A3(-/-)/ITGA2(-/-) and COL4A3(-/-)/ITGA2(+/+) mice. The double knockouts lived 20% longer and showed less glomerular and tubulointerstitial extracellular matrix deposition than the COL4A3(-/-) Alport mice with normal integrin alpha 2 beta 1 expression. Electron microscopy illustrated improvements in the glomerular basement membrane structure. MMP2, MMP9, MMP12 and TIMP1 were expressed at significantly higher levels (compared to wild-type mice) in COL4A3(-/-)/ITGA2(+/+) Alport mice, but not in COL4A3(+/+)/ITGA2(-/-) mice."

According to the news editors, the research concluded: "The collagen receptors DDR1 and integrin alpha 2 beta 1 contribute to regulate GBM-maturation and to control matrix accumulation. As demonstrated in the type IV collagen disease Alport syndrome, glomerular cell-matrix interactions via collagen receptors play an important role in the progression of renal fibrosis."

For more information on this research see: Collagen receptors integrin alpha2beta1 and discoidin domain receptor 1 regulate maturation of the glomerular basement membrane and loss of integrin alpha2betal delays kidney fibrosis in COL4A3 knockout mice. Matrix Biology, 2014;34():13-21. Matrix Biology can be contacted at: Elsevier Science Bv, PO Box 211, 1000 Ae Amsterdam, Netherlands. (Elsevier - www.elsevier.com; Matrix Biology - www.elsevier.com/wps/product/cws_home/601342)

The news correspondents report that additional information may be obtained from D. Rubel, University of Cologne, Dept. of Dermatol, Cologne, Germany. Additional authors for this research include J. Frese, M. Martin, A. Leibnitz, R. Girgert, N. Miosge, B. Eckes, G.A. Muller and O. Gross (see also Alport Syndrome).

Keywords for this news article include: Europe, Kidney, Cologne, Germany, Collagen, Integrins, Nephrology, Alport Syndrome, Membrane Proteins, Hereditary Nephritis, Immunologic Receptors, Glomerular Basement Membrane, Extracellular Matrix Proteins

Our reports deliver fact-based news of research and discoveries from around the world. Copyright 2014, NewsRx LLC


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Source: Health & Medicine Week


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