Autosomal dominant polycystic kidney disease: Disrupted pathways and potential therapeutic interventions. Issue 8 (15th January 2019)

• Record Type: Journal Article
• Authors: Malekshahabi, Talieh; Khoshdel Rad, Niloofar; Serra, Andreas L.; Moghadasali, Reza
• Is Part Of: Journal of cellular physiology. Volume 234:Issue 8(2019:Aug.)
• Page Start: 12451
• Page End: 12470
• Publication Date: 2019-01-15
• Subjects: autosomal dominant polycystic kidney disease -- cystogenesis -- end‐stage renal diseases -- pathogenesis -- polycystin 1 and 2; Physiology -- Periodicals; Cell physiology -- Periodicals; 571.6
• DOI: 10.1002/jcp.28094
• Languages: English
• ISSNs: 0021-9541

Abstract

Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic inherited renal cystic disease that occurs in different races worldwide. It is characterized by the development of a multitude of renal cysts, which leads to massive enlargement of the kidney and often to renal failure in adulthood. ADPKD is caused by a mutation in PKD1 or PKD2 genes encoding the proteins polycystin‐1 and polycystin‐2, respectively. Recent studies showed that cyst formation and growth result from deregulation of multiple cellular pathways like proliferation, apoptosis, metabolic processes, cell polarity, and immune defense. In ADPKD, intracellular cyclic adenosine monophosphate (cAMP) promotes cyst enlargement by stimulating cell proliferation and transepithelial fluid secretion. Several interventions affecting many of these defective signaling pathways have been effective in animal models and some are currently being tested in clinical trials. Moreover, the stem cell therapy can improve nephropathies and according to studies were done in this field, can be considered as a hopeful therapeutic approach in future for PKD. This study provides an in‐depth review of the relevant molecular pathways associated with the pathogenesis of ADPKD and their implications in development of potential therapeutic strategies. Abstract : Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic inherited renal cystic disease that is characterized by the development of a multitude of renal cysts, which leads to renal failure in adulthood. Recent studies showed that cyst formation and growth result from deregulation of multiple cellular pathways like proliferation, apoptosis, metabolic processes, cell polarity, and immune defense. This study provides an in‐depth review of the relevant molecular pathways associated with the pathogenesis of ADPKD and their implications in development of potential therapeutic strategies.