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多囊肾病(Polycystic kidney disease,PKD)是一种常见的严重危害人类健康的遗传疾病,以双侧肾脏肾小管形成多个进行性增大的囊肿为主要特征。高选择性运输水的跨膜通道蛋白—水通道蛋白,通过调节细胞体积和内部渗透压,在多囊肾病的发生发展中起着重要作用,其中AQP1~4的影响尤为重要。AQP位于肾脏近曲小管及亨利袢降支细段的顶质膜与侧膜上,AQP2和AQP3共同表达于集合管,约30%的肾囊肿来自于集合管,所以可以通过水通道蛋白的表达,大致定位肾囊肿的起源。肾囊泡的形成和增大主要是3个病理过程共同作用的结果即:(1)肾小管上皮细胞过度增殖;(2)囊泡内液体积聚;(3)细胞外基质改变。AQP11在多囊肾的发生过程中的意义可能更大。。
Polycystic kidney disease (PKD) is a common genetic disease that seriously endangers human health. It is mainly characterized by the formation of a plurality of progressively enlarged cysts in bilateral renal tubules. The transmembrane channel protein - aquaporin, which is highly selective transport water, plays an important role in the development of polycystic kidney disease by regulating cell volume and internal osmotic pressure. The effects of AQP1 ~ 4 are especially important. AQP is located on the apical membrane and the lateral membrane of the proximal tubule and the hemi-descending branch of the kidney. AQP2 and AQP3 are co-expressed in the collecting duct and about 30% of the renal cysts are in the collecting duct. Therefore, the expression of aquaporin , Roughly locating the origin of renal cysts. The formation and enlargement of renal vesicles are mainly the result of three pathological processes acting together: (1) tubular epithelial hyperproliferation; (2) vesicle fluid accumulation; (3) extracellular matrix changes. The significance of AQP11 in the pathogenesis of polycystic kidney disease may be greater. .