title: [From the molecular genetics of Alport's syndrome to principles of organo-protection in chronic renal diseases].
abstract: BACKGROUND
  Scarring is known to be the endpoint of most chronic kidney diseases. Therefore, prevention of renal fibrosis is a very important topic. The hereditary type IV collagen disease Alport's syndrome is a rare, but challenging cause of chronic renal fibrosis.
  Increasing knowledge about the pathogenesis of Alport's syndrome may help to find principles of nephro-protection in chronic renal diseases. The defect gene in Alport's syndrome causes an altered assembly of extracellular matrix leading to a defect cell-matrix interaction and fibrosis. This scarring is regulated by comparable mechanisms as in diabetic nephropathy or chronic inflammatory renal diseases. NEPHRO-PROTECTION IN ANIMAL MODELS: By using an Alport animal model of chronic renal fibrosis, principles of nephro-protective therapies such as blockade of the renin-angiotensin system or the effect of HMG-CoA reductase inhibitors can be investigated. CURRENT AND FUTURE NEPHRO-PROTECTION IN HUMANS: The same model serves for evaluation of new organo-protective therapies such as vasopeptidase inhibitors, blockade of endothelin, chemokine and collagen receptors as well as stem cell therapy and their potential benefit for patients with chronic renal diseases.
authors.affiliation: Medizinische Klinik I Kliniken der Stadt Köln gGmbH, Krankenhaus Merheim, Medizinische Fakultät der Universität zu Köln, Ostmerheimer Strasse 200, 51109 Köln. oliver.gross@uni-koeln.de
authors.forename: Oliver
authors.initials: O
authors.lastname: Gross
authors.forename: Manfred
authors.initials: M
authors.lastname: Weber
mesh_terms: D000818:Animals; D024141:Collagen Type IV / Q000235:genetics*; D004195:Disease Models, Animal; D050533:Glomerular Basement Membrane / Q000473:pathology; D006801:Humans; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology / Q000517:prevention & control; D051379:Mice; D008854:Microscopy, Electron; D008967:Molecular Biology; D009394:Nephritis, Hereditary / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D009395:Nephritis, Interstitial / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology / Q000517:prevention & control; D009400:Nephrosclerosis / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology / Q000517:prevention & control; D020011:Protective Agents / Q000627:therapeutic use*
chemical_list: D024141:Collagen Type IV; D020011:Protective Agents