Evidence map›Paper›PMID 28025703›Full record

ReviewCell and tissue research2017

The biology of the ABCA3 lipid transporter in lung health and disease.

Michael F Beers, Surafel Mulugeta

Abstract readReview
In one paragraph

Review in Cell and tissue research, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 72 papers.

0numbers the graph read from it
0cells of the map it votes in
72citing papers in PubMed
4.4field-weighted citation impact, top 5% 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

72 citing papers in PubMed, 117 citations in OpenAlex.

  1. Article
  2. Lentiviral-mediated gene complementation to rescue pathogenic ABCA3 variants.American journal of respiratory cell and molecular biology · 2026
    Article
  3. Article
  4. Review
  5. Review
  6. Biallelic LAMP3 variants in 5 families with interstitial lung disease: Evidence of a disease-gene association.Genetics in medicine : official journal of the American College of Medical Genetics · 2026
    Article
  7. Article
  8. Article
  9. Article
  10. Article
  11. Lentiviral-mediated gene complementation rescues pathogenicbioRxiv : the preprint server for biology · 2025
    Article
  12. Article
  13. Respirology case reports · 2025
    Article
  14. Article
  15. Novel Compound Heterozygous Mutation of theJournal of clinical medicine · 2025
    Article
  16. Origin and stepwise evolution of vertebrate lungs.Nature ecology & evolution · 2025
    Article
  17. Interstitial lung disease in the newborn.Journal of perinatology : official journal of the California Perinatal Association · 2025
    Review
  18. Article
  19. Biomedicines · 2024
    Review
  20. Case of familial interstitial lung disease attributed to ATP-binding cassette transporter 3 gene mutation in identical twins.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2024
    Article

12 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

2 authors at 1 institution in 1 country.

Michael F BeersPulmonary, Allergy, and Critical Care Division, Department of Medicine, Surfactant Biology Laboratories, University of Pennsylvania Perelman School of Medicine, Edward J. Stemmler Hall, Suite 218, 3450 Hamilton Walk, Philadelphia, PA 19104, USA.
Surafel MulugetaPulmonary, Allergy, and Critical Care Division, Department of Medicine, Surfactant Biology Laboratories, University of Pennsylvania Perelman School of Medicine, Edward J. Stemmler Hall, Suite 218, 3450 Hamilton Walk, Philadelphia, PA 19104, USA. mulugeta@mail.med.upenn.edu.
University of Pennsylvania · US

Funding

Translational Research Support CoreP30ES013508 · NIEHS · UNIVERSITY OF PENNSYLVANIA · PI Rebecca A Simmons · 2006 to 2026
$35.3M
ABCA3: Biosynthesis, Trafficking, and Cellular Responses in Health and DiseaseR01HL090732 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI MULUGETA, SURAFEL · 2008 to 2012
$2.0M
Biosynthesis and Trafficking of Surfactant Protein C In Health and DiseaseR01HL119436 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI BEERS, MICHAEL FRANCIS · 2013 to 2017
$2.0M
The Biology of the ABCA3 Lipid Transporter in Health and DiseaseR01HL129150 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI MULUGETA, SURAFEL · 2016 to 2019
$1.9M
Surfactant Protein C Mutations:Interstitial Lung DiseaseR01HL074064 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI BEERS, MICHAEL FRANCIS · 2003 to 2006
$1.8M
Surfactant Protein C Mutations and Interstitial Lung DiseaseI01BX001176 · VA · PHILADELPHIA VA MEDICAL CENTER · PI MICHAEL FRANCIS BEERS · 2012 to 2026
–
BLRD VA I01 BX001176NHLBI NIH HHS R01 HL074064NHLBI NIH HHS R01 HL090732NHLBI NIH HHS R01 HL119436NHLBI NIH HHS R01 HL129150NIEHS NIH HHS P30 ES013508
6 · The paper itself

Abstract

The lipid transporter, ATP-binding cassette class A3 (ABCA3), is a highly conserved multi-membrane-spanning protein that plays a critical role in the regulation of pulmonary surfactant homeostasis. Mutations in ABCA3 have been increasingly recognized as one of the causes of inherited pulmonary diseases. These monogenic disorders produce familial lung abnormalities with pathological presentations ranging from neonatal surfactant-deficiency-induced respiratory failure to childhood or adult diffuse parenchymal lung diseases for which specific treatment modalities remain limited. More than 200 ABCA3 mutations have been reported to date with approximately three quarters of patients presenting as compound heterozygotes. Recent advances in our understanding of the molecular basis underlying normal ABCA3 biosynthesis and processing and of the mechanisms of alveolar epithelial cell dysregulation caused by the expression of its mutant forms are beginning to emerge. These insights and the role of environmental factors and modifier genes are discussed in the context of the considerable variability in disease presentation observed in patients with identical ABCA3 gene mutations. Moreover, the opportunities afforded by an enhanced understanding of ABCA3 biology for targeted therapeutic strategies are addressed.

Indexed as

HealthAnimalsATP-Binding Cassette TransportersBiological TransportHumansLipidsLungLung DiseasesSurface-Active AgentsATP-Binding Cassette TransportersLipidsSurface-Active AgentsABCA3Compound heterozygous mutationsInhertited pulmonary diseasePulmonary surfactantSurfactant deficiency

Identifiers

PMID28025703
PMCPMC5321817
OpenAlexW2562335987

What OpenQuestion holds

Textmetadata
LicenceTDM
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.