ABSTRACT
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of renal insufficiency. It is caused by mutations to the PKD1 or PKD2 gene and is characterized by accumulation of renal cysts that ultimately lead to loss of renal function and renal failure, accounting for 5%10% of overall end-stage renal disease. The treatment options for management of this disease remain largely supportive. Thus, understanding the pathophysiology of ADPKD is imperative for the development of newer effective therapies for this genetic disorder. Recent studies indicate a central role of metabolic sensors and energy metabolism in the pathogenesis of this cystic disease. Despite advances, there are still gaps in our understanding of metabolic sensors and their role in the pathogenesis of ADPKD. Therefore, the mechanisms that are involved in these metabolic alterations and through which the metabolic sensors and nutritional manipulations interact with the metabolic and signaling pathways are important to study. In this chapter, we review the role of energy metabolism and metabolic sensors, as well as nutritional manipulations in ADPKD pathogenesis, further discussing the possible tools and approaches that can be utilized in future studies involving these interventions.
