Microvascular aberrations found in human polycystic kidneys are an early feature in a Pkd1 mutant mouse model.

ABSTRACT: Therapies targeting blood vessels hold promise for autosomal dominant polycystic kidney disease (ADPKD), the most common inherited disorder causing kidney failure. However, the onset and nature of kidney vascular abnormalities in ADPKD are poorly defined. Accordingly, we employed a combination of single-cell transcriptomics; three-dimensional imaging with geometric, topological and fractal analyses; and multimodal magnetic resonance imaging with arterial spin labelling to investigate aberrant microvasculature in ADPKD kidneys. Within human ADPKD kidneys with advanced cystic pathology and excretory failure, we identified a molecularly distinct blood microvascular subpopulation, characterised by impaired angiogenic signalling and metabolic dysfunction, differing from endothelial injury profi

SUBMITTER: Jafree DJ 

PROVIDER: S-EPMC12067086 | biostudies-literature | 2025 Apr

REPOSITORIES: biostudies-literature