{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T19:26:55Z","timestamp":1787167615190,"version":"build-2736575974"},"reference-count":56,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2011,6,27]],"date-time":"2011-06-27T00:00:00Z","timestamp":1309132800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2011,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    In autosomal dominant polycystic kidney disease, cysts arise focally and disrupt normal renal tissue leading to renal failure. In the present study, we show that cyst\u2010lining cells express the stem cell marker CD133. CD133+ progenitor cells isolated from polycystic kidney, carrying mutations of\n                    <jats:italic>PKD<\/jats:italic>\n                    genes, showed a dedifferentiated phenotype similar to CD133+ progenitor cells from normal kidney. However, these cells were more proliferative and presented a defective epithelial differentiation phenotype with respect to normal renal CD133+ cells as they were not able to express all tubular epithelial cell markers when cultured in epithelial differentiation medium. Polycystic CD133+ cells, in contrast to normal renal CD133+ cells, formed cysts\n                    <jats:italic>in vitro<\/jats:italic>\n                    in a three\u2010dimensional culture system and\n                    <jats:italic>in vivo<\/jats:italic>\n                    when injected subcutaneously within Matrigel in SCID mice. Rapamycin treatment reduced\n                    <jats:italic>in vitro<\/jats:italic>\n                    proliferation of polycystic CD133+ cells and decreased cystogenesis both\n                    <jats:italic>in vitro<\/jats:italic>\n                    and\n                    <jats:italic>in vivo.<\/jats:italic>\n                    The\n                    <jats:italic>in vitro<\/jats:italic>\n                    epithelial differentiation was only partially improved by rapamycin. These results indicate that polycystic CD133+ cells retain a dedifferentiated phenotype and the ability to generate cysts. Copyright \u00a9 2011 Pathological Society of Great Britain and Ireland. 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