Evidence map›Paper›PMID 17099139›Full record

ReviewAmerican journal of respiratory cell and molecular biology2007

Molecular pathogenesis of lymphangioleiomyomatosis: lessons learned from orphans.

Stephen C Juvet, Francis X McCormack, David J Kwiatkowski, Gregory P Downey

Abstract readReview
In one paragraph

Review in American journal of respiratory cell and molecular biology, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
41citing papers in PubMed, 1 pooled it
–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

41 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2025
    Guideline
  2. Trial
  3. Article
  4. Article
  5. Review
  6. Article
  7. Article
  8. "T"eeing Up A Novel Therapy for Lymphangioleiomyomatosis.American journal of respiratory cell and molecular biology · 2020
    Article
  9. Observational
  10. Article
  11. Article
  12. Article
  13. Review
  14. Article
  15. Multiple cystic lung disease.European respiratory review : an official journal of the European Respiratory Society · 2015
    Review
  16. Article
  17. Article
  18. Article
  19. Article
  20. Review
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.

Stephen C JuvetNational Jewish Medical and Research Center, 1400 Jackson Street, Denver, CO 80206, USA.
Francis X McCormack
David J Kwiatkowski
Gregory P Downey

Funding

RARE LUNG DISEASE CLINICAL RESEARCH NETWORKU54RR019498 · NCRR · CINCINNATI CHILDRENS HOSP MED CTR · PI TRAPNELL, BRUCE C · 2003 to 2008
$5.7M
Tuberous Sclerosis: Mutations and Murine ModelsR37NS031535 · NINDS · BRIGHAM AND WOMEN'S HOSPITAL · PI KWIATKOWSKI, DAVID J. · 2007 to 2013
$2.5M
TUBEROUS SCLEROSIS--MUTATIONS AND MURINE MODELSR01NS031535 · NINDS · BRIGHAM AND WOMEN'S HOSPITAL · PI KWIATKOWSKI, DAVID J. · 1994 to 2006
$2.3M
NCRR NIH HHS U54 RR019498NINDS NIH HHS NS031535
6 · The paper itself

Abstract

Lymphangioleiomyomatosis (LAM) is a rare progressive cystic lung disease affecting young women. The pivotal observation that LAM occurs both spontaneously and as part of the tuberous sclerosis complex (TSC) led to the hypothesis that these disorders share common genetic and pathogenetic mechanisms. In this review we describe the evolution of our understanding of the molecular and cellular basis of LAM and TSC, beginning with the discovery of the TSC1 and TSC2 genes and the demonstration of their involvement in sporadic (non-TSC) LAM. This was followed by rapid delineation of the signaling pathways in Drosophila melanogaster with confirmation in mice and humans. This knowledge served as the foundation for novel therapeutic approaches that are currently being used in human clinical trials.

Indexed as

AnimalsDrosophila melanogasterHumansLung NeoplasmsLymphangioleiomyomatosisMiceModels, BiologicalMyocytes, Smooth MuscleSignal TransductionTuberous SclerosisTuberous Sclerosis Complex 1 ProteinTuberous Sclerosis Complex 2 ProteinTumor Suppressor ProteinsTSC1 protein, humanTsc1 protein, mouseTSC2 protein, humanTsc2 protein, mouseTuberous Sclerosis Complex 1 ProteinTuberous Sclerosis Complex 2 ProteinTumor Suppressor Proteins

Identifiers

PMID17099139
PMCPMC2176113

What OpenQuestion holds

Textmetadata
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.