[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.

PATHOGENESIS OF GLOMERULAR AND INTERSTITIAL RENAL FIBROSIS IN HUMANS:

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: Oliver Gross, Manfred Weber
• Journal: Medizinische Klinik (Munich, Germany : 1983) (Med Klin (Munich)) Vol. 100 Issue 12 Pg. 826-31 (Dec 15 2005) ISSN: 0723-5003 [Print] Germany
• Vernacular Title: Von der Molekulargenetik des Alport-Syndroms zu Prinzipien der Organprotektion bei chronischen Nierenerkrankungen.
• PMID: 16453099 (Publication Type: English Abstract, Journal Article)

Chemical References

• Collagen Type IV
• Protective Agents

Topics

• Animals
• Collagen Type IV (genetics)
• Disease Models, Animal
• Glomerular Basement Membrane (pathology)
• Humans
• Kidney Failure, Chronic (diagnosis, genetics, pathology, prevention & control)
• Mice
• Microscopy, Electron
• Molecular Biology
• Nephritis, Hereditary (diagnosis, genetics, pathology)
• Nephritis, Interstitial (diagnosis, genetics, pathology, prevention & control)
• Nephrosclerosis (diagnosis, genetics, pathology, prevention & control)
• Protective Agents (therapeutic use)