Polycystic kidney disease

Tissues and organoids derived from human pluripotent stem cells with knocked-out kinesin-2 subunits lack cilia, and can be used to model ciliopathy phenotypes and to reveal underlying mechanisms of disease.

Daneshgar et al. investigated the role of mitochondrial dysfunction in ADPKD and mitochondria protective agents as potential therapeutics. The authors reported decreased mitochondrial complex activity and downregulation of mitochondrial and metabolic proteins in ADPKD, and proposed mCAT overexpression or SS31 treatment to slow cystogenesis.

Re-expression of Pkd genes in cystic kidneys results in rapid reversal of autosomal dominant polycystic kidney disease phenotypes in mice, revealing an unexpected capacity for renal plasticity under the control of Pkd gene function.