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This involves active, uphill transcellular Cl<jats:sup>\u2212<\/jats:sup> transport which drives passive Na<jats:sup>+<\/jats:sup> and water secretion. Implicit in this mechanism is the assumption that the paracellular permeability of the cyst epithelium to Cl<jats:sup>\u2212<\/jats:sup> must be very low. Claudins are tight junction (TJ) transmembrane proteins that determine the ion selectivity of paracellular barriers. The aim of this study was to determine the expression and localization of claudins within renal cysts in a mouse hypomorphic model of ADPKD and in human patients. We found that the majority of cysts were of collecting duct origin. Claudins normally expressed in collecting duct (3, 4, 7, 8, and 10) were found in small cysts. However, only claudin\u20107 persisted at substantive levels in the dedifferentiated epithelium of large, presumably late\u2010stage cysts, where it was localized both at the TJ and basolaterally. The constitutively expressed TJ proteins, ZO\u20101 and occludin, were also abundantly expressed and correctly localized, suggesting that the basic infrastructure of the TJ is preserved. A previous study suggested that claudin\u20107 may function as a paracellular Cl<jats:sup>\u2212<\/jats:sup> barrier. We postulate that the role of claudin\u20107 in ADPKD is to seal the paracellular route in Cl<jats:sup>\u2212<\/jats:sup>\u2010secreting cyst epithelium, preventing backleak of Cl<jats:sup>\u2212<\/jats:sup>, and that it thereby plays a permissive role in fluid secretion and cyst growth. Copyright \u00a9 2008 Pathological Society of Great Britain and Ireland. 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