Gross Oliver, Weber Manfred
Medizinische Klinik I Kliniken der Stadt Köln gGmbH, Krankenhaus Merheim, Medizinische Fakultät der Universität zu Köln, Ostmerheimer Strasse 200, 51109 Köln.
Med Klin (Munich). 2005 Dec 15;100(12):826-31. doi: 10.1007/s00063-005-1114-1.
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.
瘢痕形成是大多数慢性肾脏疾病的终点。因此，预防肾纤维化是一个非常重要的课题。遗传性IV型胶原病——阿尔波特综合征是慢性肾纤维化的一个罕见但具有挑战性的病因。
对阿尔波特综合征发病机制的了解不断增加，可能有助于找到慢性肾脏疾病中肾脏保护的原则。阿尔波特综合征中的缺陷基因导致细胞外基质组装改变，从而导致细胞 - 基质相互作用缺陷和纤维化。这种瘢痕形成受与糖尿病肾病或慢性炎症性肾脏疾病类似的机制调控。
通过使用慢性肾纤维化的阿尔波特动物模型，可以研究肾脏保护疗法的原则，如肾素 - 血管紧张素系统阻断或HMG - CoA还原酶抑制剂的作用。
同一模型可用于评估新的器官保护疗法，如血管肽酶抑制剂、内皮素、趋化因子和胶原受体阻断，以及干细胞疗法及其对慢性肾脏疾病患者的潜在益处。
