Abstract
Autosomal dominant (AD) and autosomal recessive (AR) polycystic kidney diseases (PKD) are severe multisystem genetic disorders characterized with formation and uncontrolled growth of fluid-filled cysts in the kidney, the spread of which eventually leads to the loss of renal function. Currently, there are no treatments for ARPKD, and tolvaptan is the only FDA-approved drug that alleviates the symptoms of ADPKD. However, tolvaptan has only a modest effect on disease progression, and its long-term use is associated with many side effects. Therefore, there is still a pressing need to better understand the fundamental mechanisms behind PKD development. This review highlights current knowledge about the fundamental aspects of PKD development (with a focus on ADPKD) including the PC1/PC2 pathways and cilia-associated mechanisms, major molecular cascades related to metabolism, mitochondrial bioenergetics, and systemic responses (hormonal status, levels of growth factors, immune system, and microbiome) that affect its progression. In addition, we discuss new information regarding non-pharmacological therapies, such as dietary restrictions, which can potentially alleviate PKD.
Highlights
One of the most common inherited disorders in the United States is polycystic kidney disease (PKD), which is characterized by the formation of fluid-filled cysts in the kidneys
This review introduces the reader to selected classic molecular mechanisms underlying polycystic kidney diseases (PKD), such as PC1/2 signaling, cilia-related cascades, and growth factors-related signaling
Despite the availability of tolvaptan for ADPKD, it is imperative to keep searching for new, side-effect free treatments that would reduce the suffering of the PKD patients
Summary
One of the most common inherited disorders in the United States is polycystic kidney disease (PKD), which is characterized by the formation of fluid-filled cysts in the kidneys. There is a possibility that dysregulation of RAAS can potentially contribute to disease progression by activating signaling cascades that promote cyst growth and especially affect epithelial transport.
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