Evidence map›Paper›PMID 37400371›Full record

ArticleAdvanced science (Weinheim, Baden-Wurttemberg, Germany)2023

Tissue-Engineered Disease Modeling of Lymphangioleiomyomatosis Exposes a Therapeutic Vulnerability to HDAC Inhibition.

Adam Pietrobon, Julien Yockell-Lelièvre, Nicole Melong, Laura J Smith, Sean P Delaney, Nadine Azzam, Chang Xue, Nishanth Merwin, Eric Lian, Alberto Camacho-Magallanes and 8 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Advanced science (Weinheim, Baden-Wurttemberg, Germany), 2023. 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. Current insight into the molecular mechanisms of lymphangioleiomyomatosis.American journal of respiratory cell and molecular biology · 2026
    Review
  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

18 authors.

Adam PietrobonThe Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, K1Y 4E9, Canada.
Julien Yockell-LelièvreThe Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, K1Y 4E9, Canada.
Nicole MelongDepartment of Pediatrics, CHEO Research Institute, Ottawa, K1H 5B2, Canada.
Laura J SmithDepartment of Chemical Engineering and Applied Chemistry, University of Toronto, Toronto, M5S 3E5, Canada.
Sean P DelaneyThe Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, K1Y 4E9, Canada.
Nadine AzzamDepartment of Pediatrics, CHEO Research Institute, Ottawa, K1H 5B2, Canada.
Chang XueInstitute for Biomaterials and Biomedical Engineering, University of Toronto, Toronto, M5S 3G9, Canada.
Nishanth MerwinBioSymetrics, Inc., Toronto, M5T 1X5, Canada.
Eric LianThe Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, K1Y 4E9, Canada.
Alberto Camacho-MagallanesThe Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, K1Y 4E9, Canada.
Carole DoréThe Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, K1Y 4E9, Canada.
Gabriel MussoBioSymetrics, Inc., Toronto, M5T 1X5, Canada.
Lisa M JulianCentre for Cell Biology, Development, and Disease, Department of Biological Sciences, Simon Fraser University, Burnaby, V5A 1S6, Canada.
Arnold S KristofMeakins-Christie Laboratories and Translational Research in Respiratory Diseases Program, Research Institute of the McGill University Health Centre, Faculty of Medicine, Departments of Medicine and Critical Care, Montreal, H4A 3J1, Canada.
Roger Y TamCentre for Biologics Evaluation, Biologic and Radiopharmaceutical Drugs Directorate, Health Canada, Ottawa, K1Y 4X2, Canada.
Jason N BermanDepartment of Cellular and Molecular Medicine, University of Ottawa, Ottawa, K1N 6N5, Canada.
Molly S ShoichetDepartment of Chemical Engineering and Applied Chemistry, University of Toronto, Toronto, M5S 3E5, Canada.
William L StanfordThe Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, K1Y 4E9, Canada.ORCID 0000-0002-5813-8016

Funding

Canada Research ChairsCanadian Institutes for Health Research 153188CIHR Vanier Canada Graduate ScholarshipLAM Foundation LAM0123P01-17Ontario Institute for Cancer Research
6 · The paper itself

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease involving cystic lung destruction by invasive LAM cells. These cells harbor loss-of-function mutations in TSC2, conferring hyperactive mTORC1 signaling. Here, tissue engineering tools are employed to model LAM and identify new therapeutic candidates. Biomimetic hydrogel culture of LAM cells is found to recapitulate the molecular and phenotypic characteristics of human disease more faithfully than culture on plastic. A 3D drug screen is conducted, identifying histone deacetylase (HDAC) inhibitors as anti-invasive agents that are also selectively cytotoxic toward TSC2

Indexed as

Lung NeoplasmsLymphangioleiomyomatosisAnimalsHistone Deacetylase InhibitorsHumansMechanistic Target of Rapamycin Complex 1Tissue EngineeringZebrafishHistone Deacetylase InhibitorsMechanistic Target of Rapamycin Complex 13D drug screenbiomimetic hydrogel cultureHDAC inhibitionlymphangioleiomyomatosismTORC1therapeutics developmentzebrafish

Identifiers

PMID37400371
PMCPMC10502849

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.