The PAX2 tanscription factor is expressed in cystic and hyperproliferative dysplastic epithelia in human kidney malformations.

作者: P J Winyard , R A Risdon , V R Sams , G R Dressler , A S Woolf

DOI: 10.1172/JCI118811

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摘要: Human dysplastic kidneys are developmental aberrations which responsible for many of the very young children with chronic renal failure. They contain poorly differentiated metanephric cells in addition to metaplastic elements. We recently demonstrated that apoptosis was prominent undifferentiated around tubules (Winyard, P.J.D., J. Nauta, D.S. Lirenman, P. Hardman, V.R. Sams, R.A. Risdon, and A.S. Woolf. 1996. Kidney Int. 49:135-146), perhaps explaining tendency some these organs regress. In contrast, rare epithelia thought be ureteric bud malformations. On occasion, form cysts distend abdominal cavity (the multicystic kidney) may rarely become malignant. now demonstrate maintain a high rate proliferation postnatally PAX2, potentially oncogenic transcription factor, is expressed epithelia. both cell PAX2 downregulated during normal maturation human collecting ducts. BCL2, protein prevents mesenchymal epithelia] conversion, ectopically kidney propose cyst formation understood terms aberrant temporal spatial expression master genes tightly regulated program nephrogenesis.

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