Diagnosis, pathogenesis, and treatment prospects in cystic kidney disease

Carsten Bergmann*, Valeska Frank, Fabian Küpper, Dirk Kamitz, Jens Hanten, Peter Berges, Silke Mager, Markus Moser, Jutta Kirfel, Reinhard Büttner, Jan Senderek, Klaus Zerres

*Corresponding author for this work
12 Citations (Scopus)


Cystic kidney diseases (CKDs) are a clinically and genetically heterogeneous group of disorders characterized by progressive fibrocystic renal and hepatobiliary changes. Recent findings have proven the cystogenic process to be compatible with cellular dedifferentiation, i. e. increased apoptosis and proliferation rates, altered protein sorting and secretory characteristics, as well as disorganization of the extracellular matrix. Compelling evidence suggests that cilia play a central pathogenic role and most cystic kidney disorders converge into a common pathogenic pathway. Recently, several promising trials have further extended our understanding of the pathophysiology of CKD and may have the potential for rational personalized therapies in future years. This review aims to summarize the current state of knowledge of the structure and function of proteins underlying polycystic kidney disease, to explore the clinical consequences of changes in respective genes, and to discuss potential therapeutic approaches.

Original languageEnglish
JournalMolecular Diagnosis and Therapy
Issue number3
Pages (from-to)163-174
Number of pages12
Publication statusPublished - 01.01.2006


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