Evidence map›Paper›PMID 23268535›Full record

ReviewDisease models & mechanisms2013

Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis.

Jonathan A Kropski, William E Lawson, Lisa R Young, Timothy S Blackwell

Open access · goldAbstract readCase ReportsReview
In one paragraph

Review in Disease models & mechanisms, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 76 papers, 3 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
76citing papers in PubMed, 3 pooled it
14.4field-weighted citation impact, top 1% 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

76 citing papers in PubMed, 3 syntheses or guidelines pooled it, 153 citations in OpenAlex.

  1. Pooled it
  2. Guideline
  3. Pooled it
  4. Review
  5. Article
  6. The intersection of liver cirrhosis and pulmonary fibrosis.Journal of translational medicine · 2025
    Review
  7. Article
  8. Review
  9. Review
  10. Article
  11. Genetic Underpinnings of Pulmonary Fibrosis: An Overview.Cardiovascular & hematological agents in medicinal chemistry · 2024
    Review
  12. Observational
  13. Article
  14. Article
  15. Review
  16. Review
  17. Insights into the Pathogenesis of Pulmonary Fibrosis from Genetic Diseases.American journal of respiratory cell and molecular biology · 2022
    Review
  18. Review
  19. Review
  20. Epigenetics alternation in lung fibrosis and lung cancer.Frontiers in cell and developmental biology · 2022
    Review

16 more citing papers are in PubMed but not listed here.

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

4 authors at 2 institutions in 1 country.

Jonathan A KropskiDepartment of Medicine, Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, TN 37232, USA. jon.kropski@vanderbilt.edu
William E Lawson
Lisa R Young
Timothy S Blackwell
Pulmonary and Allergy Associates · USVanderbilt University · US

Funding

Mechanisms of Familial Pulmonary FibrosisP01HL092870 · NHLBI · VANDERBILT UNIVERSITY MEDICAL CENTER · PI SCHWARTZ, DAVID ALBERT · 2010 to 2020
$21.6M
Interdisciplinary Training Program in Lung ResearchT32HL094296 · NHLBI · VANDERBILT UNIVERSITY MEDICAL CENTER · PI Ray Stokes Peebles · 2008 to 2026
$6.3M
Mechanisms of Pulmonary Fibrosis in Hermansky-Pudlak SyndromeR01HL119503 · NHLBI · VANDERBILT UNIVERSITY MEDICAL CENTER · PI YOUNG, LISA R. · 2013 to 2021
$3.6M
Epithelial-Fibroblast Interactions in Lung FibrosisR01HL085317 · NHLBI · VANDERBILT UNIVERSITY · PI BLACKWELL, TIMOTHY S. · 2007 to 2015
$3.5M
Regulation of Fibrotic Remodeling by the Alveolar EpitheliumR01HL105479 · NHLBI · VANDERBILT UNIVERSITY MEDICAL CENTER · PI LAWSON, WILLIAM E · 2011 to 2015
$2.2M
Alveolar Cell Dysfunction and Pulmonary FibrosisK08HL082757 · NHLBI · VANDERBILT UNIVERSITY · PI YOUNG, LISA R. · 2007 to 2011
$640k
NHLBI NIH HHS HL082757NHLBI NIH HHS HL085317NHLBI NIH HHS HL092870NHLBI NIH HHS HL094296NHLBI NIH HHS HL105479NHLBI NIH HHS K08 HL082757NHLBI NIH HHS P01 HL092870NHLBI NIH HHS R01 HL085317NHLBI NIH HHS R01 HL105479NHLBI NIH HHS R01 HL119503NHLBI NIH HHS T32 HL094296
6 · The paper itself

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and often fatal lung disease for which there is no known treatment. Although the traditional paradigm of IPF pathogenesis emphasized chronic inflammation as the primary driver of fibrotic remodeling, more recent insights have challenged this view. Linkage analysis and candidate gene approaches have identified four genes that cause the inherited form of IPF, familial interstitial pneumonia (FIP). These four genes encode two surfactant proteins, surfactant protein C (encoded by SFTPC) and surfactant protein A2 (SFTPA2), and two components of the telomerase complex, telomerase reverse transcriptase (TERT) and the RNA component of telomerase (TERC). In this review, we discuss how investigating these mutations, as well as genetic variants identified in other inherited disorders associated with pulmonary fibrosis, are providing new insights into the pathogenesis of common idiopathic interstitial lung diseases, particularly IPF. Studies in this area have highlighted key roles for epithelial cell injury and dysfunction in the development of lung fibrosis. In addition, genetic approaches have uncovered the importance of several processes - including endoplasmic reticulum stress and the unfolded protein response, DNA-damage and -repair pathways, and cellular senescence - that might provide new therapeutic targets in fibrotic lung diseases.

Indexed as

Cellular SenescenceDNA DamageDNA RepairEndoplasmic Reticulum StressGenetic VariationHermanski-Pudlak SyndromeHumansIdiopathic Pulmonary FibrosisMaleMiddle AgedModels, GeneticMucin-5BMutationPulmonary Surfactant-Associated Protein APulmonary Surfactant-Associated Protein CRNAMUC5B protein, humanMucin-5BPulmonary Surfactant-Associated Protein APulmonary Surfactant-Associated Protein CRNASFTPA2 protein, humanSFTPC protein, humanTelomerasetelomerase RNATERT protein, human

Identifiers

PMID23268535
PMCPMC3529334
OpenAlexW2159036727

What OpenQuestion holds

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LicenceCC BY-NC-SA
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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.