Evidence map›Paper›PMID 31822676›Full record

ArticleNature communications2019

Monkeys mutant for PKD1 recapitulate human autosomal dominant polycystic kidney disease.

Tomoyuki Tsukiyama, Kenichi Kobayashi, Masataka Nakaya, Chizuru Iwatani, Yasunari Seita, Hideaki Tsuchiya, Jun Matsushita, Kahoru Kitajima, Ikuo Kawamoto, Takahiro Nakagawa and 11 more

Open access · goldAbstract read
In one paragraph

Article in Nature communications, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers.

0numbers the graph read from it
0cells of the map it votes in
36citing papers in PubMed
3.8field-weighted citation impact, top 6% of its field
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

36 citing papers in PubMed, 54 citations in OpenAlex.

  1. Frameshift Mutations in Exon 1 ofJournal of developmental biology · 2026
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  15. Genes · 2023
    Article
  16. Mechanisms of Cyst Development in Polycystic Kidney Disease.Advances in kidney disease and health · 2023
    Review
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  18. Article
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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

21 authors at 4 institutions in 1 country.

Tomoyuki TsukiyamaDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan. ttsuki@belle.shiga-med.ac.jp.ORCID http://orcid.org/0000-0002-2657-7719
Kenichi KobayashiDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Masataka NakayaDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Chizuru IwataniDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Yasunari SeitaDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Hideaki TsuchiyaDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Jun MatsushitaDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Kahoru KitajimaDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Ikuo KawamotoDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Takahiro NakagawaDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Koji FukudaShin Nippon Biomedical Laboratories, Ltd, Kagoshima, 891-1394, Japan.
Teppei IwakiriShin Nippon Biomedical Laboratories, Ltd, Kagoshima, 891-1394, Japan.
Hiroyuki IzumiShin Nippon Biomedical Laboratories, Ltd, Kagoshima, 891-1394, Japan.
Iori ItagakiDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Shinji KumeDepartment of Medicine, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Hiroshi MaegawaDepartment of Medicine, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Ryuichi NishinakamuraDepartment of Kidney Development, Institute of Molecular Embryology and Genetics, Kumamoto University, Kumamoto, 860-0811, Japan.
Saori NishioDivision of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Hokkaido, 060-8648, Japan.
Shinichiro NakamuraDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Akihiro KawauchiDepartment of Urology, Shiga University of Medical Science, Shiga, 520-2192, Japan.
Masatsugu EmaDepartment of Stem Cells and Human Disease Models, Research Center for Animal Life Science, Shiga University of Medical Science, Shiga, 520-2192, Japan. mema@belle.shiga-med.ac.jp.ORCID http://orcid.org/0000-0003-0645-6183
Shiga University of Medical Science · JPShin Nippon Biomedical Laboratories (Japan) · JPHokkaido University · JPKumamoto University · JP

Funding

Research BaseP30DK090868 · NIDDK · UNIVERSITY OF MARYLAND BALTIMORE · PI WATNICK, TERRY J · 2010 to 2019
$11.1M
NIDDK NIH HHS P30 DK090868
6 · The paper itself

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) caused by PKD1 mutations is one of the most common hereditary disorders. However, the key pathological processes underlying cyst development and exacerbation in pre-symptomatic stages remain unknown, because rodent models do not recapitulate critical disease phenotypes, including disease onset in heterozygotes. Here, using CRISPR/Cas9, we generate ADPKD models with PKD1 mutations in cynomolgus monkeys. As in humans and mice, near-complete PKD1 depletion induces severe cyst formation mainly in collecting ducts. Importantly, unlike in mice, PKD1 heterozygote monkeys exhibit cyst formation perinatally in distal tubules, possibly reflecting the initial pathology in humans. Many monkeys in these models survive after cyst formation, and cysts progress with age. Furthermore, we succeed in generating selective heterozygous mutations using allele-specific targeting. We propose that our models elucidate the onset and progression of ADPKD, which will serve as a critical basis for establishing new therapeutic strategies, including drug treatments.

Indexed as

Macaca fascicularisAllelesAnimalsDisease Models, AnimalFemaleHeterozygoteHumansKidneyMaleMutationPolycystic Kidney, Autosomal DominantTRPP Cation ChannelsTRPP Cation Channels

Identifiers

PMID31822676
PMCPMC6904451
OpenAlexW2996373692

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

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.