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10.1007/s00467-016-3385-6

http://scihub22266oqcxt.onion/10.1007/s00467-016-3385-6
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C5081278!5081278!27115886
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suck abstract from ncbi


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pmid27115886      Pediatr+Nephrol 2017 ; 32 (4): 557-64
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  • Role of Renal Urothelium in the Development and Progression of Kidney Disease #MMPMID27115886
  • Carpenter AR; McHugh KM
  • Pediatr Nephrol 2017[Apr]; 32 (4): 557-64 PMID27115886show ga
  • The clinical and financial impact of chronic kidney disease (CKD) is significant, while the progression and prognosis of CKD is variable and often poor. Studies using the megabladder (mgb?/?) model of CKD have shown that renal urothelium plays a key role in modulating the early injury responses following the development of congenital obstruction. The aim of this review is to examine the role that urothelium has in normal urinary tract development and pathogenesis. We discuss normal morphology of renal urothelium and then examine the role that uroplakins (Upks) play in its development. Histologic, biochemical and molecular characterization of Upk1bRFP/RFP mice indicated Upk1b expression is essential for normal urinary tract development, apical plaque/AUM formation and differentiation and functional integrity of the renal urothelium. Our studies provide the first evidence Upk1b is directly associated with the development of congenital anomalies of the urinary tract (CAKUT), spontaneous age-dependent hydronephrosis and dysplastic urothelia. These observations demonstrate the importance of proper urothelial differentiation in the normal development and pathogenesis of the urinary tract, and provide a unique working model to test the hypothesis that the complex etiology associated with CKD is dependent upon predetermined genetic susceptibilities that establish pathogenic thresholds for disease initiation and progression.
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