Evidence map›Paper›PMID 40114603›Full record

ArticleDisease models & mechanisms2025

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

Daniyal J Jafree, Charith Perera, Mary Ball, Daniele Tolomeo, Gideon Pomeranz, Laura Wilson, Benjamin Davis, William J Mason, Eva Maria Funk, Maria Kolatsi-Joannou and 22 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Disease models & mechanisms, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Renal blood flow and progression to kidney failure in ADPKD.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2026
    Observational
  2. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

32 authors.

Daniyal J JafreeDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Charith PereraUCL Centre for Advanced Biomedical Imaging, University College London, London WC1E 6DD, UK.
Mary BallDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Daniele TolomeoUCL Centre for Advanced Biomedical Imaging, University College London, London WC1E 6DD, UK.
Gideon PomeranzDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Laura WilsonDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Benjamin DavisCentral Laser Facility, Science and Technologies Facilities Council, UK Research and Innovation, Didcot OX11 0QX, UK.
William J MasonDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Eva Maria FunkDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Maria Kolatsi-JoannouDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Radu PolschiDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Saif MalikDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Benjamin J StewartMolecular Immunity Unit, Department of Medicine, University of Cambridge, Cambridge CB2 1TN, UK.
Karen L PriceDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Hannah MitchellMathematical Sciences Research Centre, Queen's University Belfast, Belfast BT7 1NN, UK.
Reza MotallebzadehUCL Centre for Kidney and Bladder Health, University College London, London WC1E 6BT, UK.
Yoshiharu MutoDivision of Nephrology, Department of Medicine, Washington University in St Louis, St Louis, MO 63110, USA.
Robert LeesCentral Laser Facility, Science and Technologies Facilities Council, UK Research and Innovation, Didcot OX11 0QX, UK.
Sarah NeedhamCentral Laser Facility, Science and Technologies Facilities Council, UK Research and Innovation, Didcot OX11 0QX, UK.
Dale MouldingDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Jennie C ChandlerDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Sonal NandanwarDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Claire L WalshUCL Centre for Advanced Biomedical Imaging, University College London, London WC1E 6DD, UK.
Paul J D WinyardDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Peter J ScamblerDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
René HägerlingLymphovascular Medicine and Translational 3D-Histopathology Research Group, Charité Universitätsmedizin Berlin, Berlin 10117, Germany.
Menna R ClatworthyMolecular Immunity Unit, Department of Medicine, University of Cambridge, Cambridge CB2 1TN, UK.
Benjamin D HumphreysDivision of Nephrology, Department of Medicine, Washington University in St Louis, St Louis, MO 63110, USA.
Mark F LythgoeUCL Centre for Advanced Biomedical Imaging, University College London, London WC1E 6DD, UK.
Simon Walker-SamuelUCL Centre for Advanced Biomedical Imaging, University College London, London WC1E 6DD, UK.
Adrian S WoolfSchool of Biological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester M13 9PT, UK.
David A LongDevelopmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.ORCID 0000-0001-6580-3435

Funding

Central Laser Facility, Science and Technology Facilities Council 23130027Disease Models & Mechanisms CTG-DMM23091209Disease Models and Mechanisms Conference Travel Grant CTG-DMM23091209Foulkes FoundationMedical Research Council MR/P018629/1NIHR Great Ormond Street Hospital Biomedical Research CentrePolycystic Kidney Disease Charity PKD-22-01Rosetrees Trust PhD2020\100012Specialised Foundation Programme in the East of England Foundation SchoolsUniversity College LondonWellcome Trust 220895Wellcome Trust 220895/Z/20/Z)Wellcome Trust 314710/Z/24/Z
6 · The paper itself

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 profiles observed in non-cystic human kidney diseases. Next, Pkd1 mutant mouse kidneys were examined postnatally, when cystic pathology is well established, but before excretory failure. An aberrant endothelial subpopulation was also detected, concurrent with reduced cortical blood perfusion. Disorganised kidney cortical microvasculature was also present in Pkd1 mutant mouse fetal kidneys when tubular dilation begins. Thus, aberrant features of cystic kidney vasculature are harmonised between human and mouse ADPKD, supporting early targeting of the vasculature as a strategy to ameliorate ADPKD progression.

Indexed as

MicrovesselsMutationPolycystic Kidney, Autosomal DominantTRPP Cation ChannelsAnimalsDisease Models, AnimalEndothelial CellsHumansKidneyMiceTRPP Cation ChannelsMagnetic resonance imagingNephrologyPerfusionSingle-cell RNA sequencingThree-dimensional microscopyVasculature

Identifiers

PMID40114603
PMCPMC12067086

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.