Evidence map›Paper›PMID 35509004›Full record

ArticleRespiratory research2022

Progressive pulmonary fibrosis in a murine model of Hermansky-Pudlak syndrome.

Shachar Abudi-Sinreich, Steven P Bodine, Tadafumi Yokoyama, Nathanial J Tolman, Michal Tyrlik, Lauren C Testa, Chen G Han, Heidi M Dorward, Stephen M Wincovitch, Yair Anikster and 4 more

Open access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Respiratory research, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
0.8field-weighted citation impact, top 31% 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

4 citing papers in PubMed, 6 citations in OpenAlex.

  1. Article
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  3. Review
  4. 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

14 authors at 4 institutions in 2 countries.

Shachar Abudi-Sinreich *Human Biochemical Genetics Section, National Human Genome Research Institute (NHGRI), National Institute of Health (NIH), Bethesda, MD, 20892, USA.
Steven P Bodine *Human Biochemical Genetics Section, National Human Genome Research Institute (NHGRI), National Institute of Health (NIH), Bethesda, MD, 20892, USA.
Tadafumi YokoyamaHuman Biochemical Genetics Section, National Human Genome Research Institute (NHGRI), National Institute of Health (NIH), Bethesda, MD, 20892, USA.
Nathanial J TolmanUDP Translational Laboratory, NIH Undiagnosed Diseases Program, National Human Genome Research Institute (NHGRI), National Institutes of Health (NIH), Bethesda, MD, 20892, USA.
Michal TyrlikHuman Biochemical Genetics Section, National Human Genome Research Institute (NHGRI), National Institute of Health (NIH), Bethesda, MD, 20892, USA.
Lauren C TestaUDP Translational Laboratory, NIH Undiagnosed Diseases Program, National Human Genome Research Institute (NHGRI), National Institutes of Health (NIH), Bethesda, MD, 20892, USA.
Chen G HanHuman Biochemical Genetics Section, National Human Genome Research Institute (NHGRI), National Institute of Health (NIH), Bethesda, MD, 20892, USA.
Heidi M DorwardHuman Biochemical Genetics Section, National Human Genome Research Institute (NHGRI), National Institute of Health (NIH), Bethesda, MD, 20892, USA.
Stephen M WincovitchNational Human Genome Research Institute (NHGRI) Cytogenetics and Microscopy Core, National Institute of Health (NIH), Bethesda, MD, 20892, USA.
Yair AniksterThe Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
William A GahlHuman Biochemical Genetics Section, National Human Genome Research Institute (NHGRI), National Institute of Health (NIH), Bethesda, MD, 20892, USA.
Resat CinarLaboratory of Physiologic Studies, National Institute on Alcohol Abuse and Alcoholism (NIAAA), National Institute of Health (NIH), Rockville, MD, 20852, USA.
Bernadette R GochuicoHuman Biochemical Genetics Section, National Human Genome Research Institute (NHGRI), National Institute of Health (NIH), Bethesda, MD, 20892, USA.
May Christine V MalicdanHuman Biochemical Genetics Section, National Human Genome Research Institute (NHGRI), National Institute of Health (NIH), Bethesda, MD, 20892, USA. malicdanm@mail.nih.gov.ORCID http://orcid.org/0000-0003-1697-6959
National Human Genome Research Institute · USNational Institutes of Health · USTel Aviv University · ILNational Institute on Alcohol Abuse and Alcoholism · US

Funding

Human Biochemical GeneticsZIAHG000215 · NHGRI · NATIONAL HUMAN GENOME RESEARCH INSTITUTE · PI GAHL, WILLIAM · 2009 to 2025
$72.6M
Fibroproliferative mechanisms in organ fibrosisZIAAA000355 · NIAAA · NATIONAL INSTITUTE ON ALCOHOL ABUSE AND ALCOHOLISM · PI CINAR, RESAT · 2021 to 2025
$6.6M
NHGRI NIH HHS Intramural Research ProgramNIAAA NIH HHS Intramural Research Programnih office of the director Common Fund
6 · The paper itself

Abstract

backgroundHPS-1 is a genetic type of Hermansky-Pudlak syndrome (HPS) with highly penetrant pulmonary fibrosis (HPSPF), a restrictive lung disease that is similar to idiopathic pulmonary fibrosis (IPF). Hps1

methodsTo develop a murine model of HPSPF that closely mimics the progression of human pulmonary fibrosis, we investigated the pulmonary effects of systemic delivery of bleomycin in Hps1

resultsOur study revealed that systemic delivery of bleomycin induced limited, acute inflammation that resolved. The distinct inflammatory phase preceded a slow, gradually progressive fibrogenesis that was shown to be both time-dependent and dose-dependent. The fibrosis phase exhibited characteristics that better resembles human disease with focal regions of fibrosis that were predominantly found in peribronchovascular areas and in subpleural regions; central lung areas contained relatively less fibrosis.

conclusionThis model provides a preclinical tool that will allow researchers to study the mechanism of pulmonary fibrosis in HPS and provide a platform for the development of therapeutics to treat HPSPF. This method can be applied on studies of IPF or other monogenic disorders that lead to pulmonary fibrosis.

Indexed as

Hermanski-Pudlak SyndromeIdiopathic Pulmonary FibrosisAlbinismAnimalsBleomycinDisease Models, AnimalFibrosisHemorrhagic DisordersLungMiceBleomycinBleomycinInterstitial lung diseasePulmonary fibrosisRare diseaseTranslational disease models

Identifiers

PMID35509004
PMCPMC9066931
OpenAlexW4229058332

What OpenQuestion holds

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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.