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

doi:10.1242/dmm.052024

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Article

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

Disease models & mechanisms - 1 Apr 2025

Jafree Daniyal J, Perera Charith, Ball Mary, Tolomeo Daniele, Pomeranz Gideon, Wilson Laura, Davis Benjamin, Mason William J, Funk Eva Maria, Kolatsi-Joannou Maria, Polschi Radu, Malik Saif, Stewart Benjamin J, Price Karen L, Mitchell Hannah, Motallebzadeh Reza, Muto Yoshiharu, Lees Robert, Needham Sarah, Moulding Dale, Chandler Jennie C, Nandanwar Sonal, Walsh Claire L, Winyard Paul J D, Scambler Peter J, Hägerling René, Clatworthy Menna R, Humphreys Benjamin D, Lythgoe Mark F, Walker-Samuel Simon, Woolf Adrian S, Long David A

Abstract excerpt

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...

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Identifiers

PMID: 40114603
DOI: 10.1242/dmm.052024
PMCID: PMC12067086

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