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dc.contributor.authorKoupepidou, P.en
dc.contributor.authorFelekkis, Kyriacos N.en
dc.contributor.authorKränzlin, B.en
dc.contributor.authorSticht, C.en
dc.contributor.authorGretz, N.en
dc.contributor.authorConstantinou-Deltas, Constantinos D.en
dc.creatorKoupepidou, P.en
dc.creatorFelekkis, Kyriacos N.en
dc.creatorKränzlin, B.en
dc.creatorSticht, C.en
dc.creatorGretz, N.en
dc.creatorConstantinou-Deltas, Constantinos D.en
dc.description.abstractBackground: Polycystic Kidney Disease is characterized by the formation of large fluid-filled cysts that eventually destroy the renal parenchyma leading to end-stage renal failure. Although remarkable progress has been made in understanding the pathologic mechanism of the disease, the precise orchestration of the early events leading to cyst formation is still unclear. Abnormal cellular proliferation was traditionally considered to be one of the primary irregularities leading to cyst initiation and growth. Consequently, many therapeutic interventions have focused on targeting this abnormal proliferation, and some have even progressed to clinical trials. However, the role of proliferation in cyst development was primarily examined at stages where cysts are already visible in the kidneys and therefore at later stages of disease development. Methods: In this study we focused on the cystic phenotype since birth in an attempt to clarify the temporal contribution of cellular proliferation in cyst development. Using a PKD2 transgenic rat model (PKD2 (1-703)) of different ages (0-60 days after birth) we performed gene expression profiling and phenotype analysis by measuring various kidney parameters. Results: Phenotype analysis demonstrated that renal cysts appear immediately after birth in the PKD2 transgenic rat model (PKD2 (1-703)). On the other hand, abnormal proliferation occurs at later stages of the disease as identified by gene expression profiling. Interestingly, other pathways appear to be deregulated at early stages of the disease in this PKD model. Specifically, gene expression analysis demonstrated that at day 0 the RAS system is involved. This is altered at day 6, when Wnt signaling and focal adhesion pathways are affected. However, at and after 24 days, proliferation, apoptosis, altered ECM signaling and many other factors become involved. Conclusions: Our data suggest that cystogenesis precedes deregulation of proliferation-related pathways, suggesting that proliferation abnormalities may contribute in cyst growth rather than cyst formation. © 2010 Koupepidou et alen
dc.description.abstractlicensee BioMed Central Ltd.en
dc.sourceBMC Nephrologyen
dc.subjectcontrolled studyen
dc.subjectkidney polycystic diseaseen
dc.subjectdisease courseen
dc.subjectcell proliferationen
dc.subjectsignal transductionen
dc.subjectanimal experimenten
dc.subjectanimal modelen
dc.subjectanimal tissueen
dc.subjectgene expression profilingen
dc.subjectWnt proteinen
dc.subjectextracellular matrixen
dc.subjectfocal adhesion kinaseen
dc.subjectkidney cysten
dc.subjectpolycystin 2en
dc.subjecttransgenic raten
dc.subjectgene controlen
dc.subjectrenin angiotensin aldosterone systemen
dc.titleCyst formation in the PKD2 (1-703) transgenic rat precedes deregulation of proliferation-related pathwaysen
dc.description.volume11Σχολή Θετικών και Εφαρμοσμένων Επιστημών / Faculty of Pure and Applied SciencesΤμήμα Βιολογικών Επιστημών / Department of Biological Sciences
dc.description.notes<p>Cited By :8</p>en
dc.source.abbreviationBMC Nephrol.en
dc.contributor.orcidConstantinou-Deltas, Constantinos D. [0000-0001-5549-9169]

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