Evidence map›Paper›PMID 11607823›Full record

ReviewOncogene2001

Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma.

F G Barr

Abstract readReview
PubMed Publisher
In one paragraph

Review in Oncogene, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 171 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
171citing papers in PubMed, 1 pooled it
3.9field-weighted citation impact, top 6% 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

171 citing papers in PubMed, 1 synthesis or guideline pooled it, 372 citations in OpenAlex.

  1. Pooled it
  2. Trial
  3. Review
  4. Article
  5. Article
  6. Article
  7. Review
  8. Article
  9. Review
  10. Review
  11. Gene Amplification in Rhabdomyosarcoma: Lessons from a Rare Cancer.International journal of molecular sciences · 2026
    Review
  12. Epigenetic, Genetic, and Functional Germline Alterations ofCurrent issues in molecular biology · 2026
    Review
  13. Genetic architecture of craniofacial morphogenesis: roles ofFrontiers in cell and developmental biology · 2026
    Review
  14. Article
  15. Review
  16. Article
  17. Review
  18. Article
  19. Article
  20. Article

111 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

1 author at 1 institution in 1 country.

F G BarrDepartment of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 36th Street and Hamilton Walk, Philadelphia, Pennsylvania, PA 19104-6082, USA. barrfg@mail.med.upenn.edu
University of Pennsylvania · US

Funding

T(2--13) OF ALVEOLAR RHABDOMYOSARCOMAR01CA064202 · NCI · UNIVERSITY OF PENNSYLVANIA · PI BARR, FREDERIC G · 1994 to 2007
$3.1M
IRSG STUDIES OF ALVEOLAR RHABDOMYOSARCOMA GENE FUSIONSR01CA089461 · NCI · UNIVERSITY OF PENNSYLVANIA · PI BARR, FREDERIC G · 2001 to 2005
$1.3M
REGULATION OF PAX3/PAX7 EXPRESSION IN RHABDOMYOSARCOMAR01CA071838 · NCI · UNIVERSITY OF PENNSYLVANIA · PI BARR, FREDERIC G · 1997 to 2001
$475k
NCI NIH HHS CA64202NCI NIH HHS CA71838NCI NIH HHS CA89461
6 · The paper itself

Abstract

The chromosomal translocations t(2;13)(q35;q14) and t(1;13)(p36;q14) are characteristic of alveolar rhabdomyosarcoma, a pediatric soft tissue cancer related to the striated muscle lineage. These translocations rearrange PAX3 and PAX7, members of the paired box transcription factor family, and juxtapose these genes with FKHR, a member of the fork head transcription factor family. This juxtaposition generates PAX3-FKHR and PAX7-FKHR chimeric genes that are expressed as chimeric transcripts that encode chimeric proteins. The fusion proteins, which contain the PAX3/PAX7 DNA binding domain and the FKHR transcriptional activation domain, activate transcription from PAX-binding sites with higher potency than the corresponding wild-type PAX proteins. This increased function results from the insensitivity of the FKHR activation domain to inhibitory effects of N-terminal PAX3/PAX7 domains. In addition to altered function, the fusion products are expressed in ARMS tumors at higher levels than the corresponding wild-type PAX products due to two distinct mechanisms. The PAX7-FKHR fusion is overexpressed as a result of in vivo amplification while the PAX3-FKHR fusion is overexpressed due to a copy number-independent increase in transcriptional rate. Finally, though FKHR subcellular localization is regulated by an AKT-dependent pathway, the fusion proteins are resistant to these signals and show exclusively nuclear localization. Therefore, these translocations alter biological activity at the levels of protein function, gene expression, and subcellular localization with the cumulative outcome postulated to be aberrant regulation of PAX3/PAX7 target genes. This aberrant gene expression program is then hypothesized to contribute to tumorigenic behavior by impacting on the control of growth, apoptosis, differentiation and motility.

Indexed as

AnimalsDNA-Binding ProteinsForkhead Box Protein O1Forkhead Transcription FactorsGene Expression Regulation, NeoplasticHomeodomain ProteinsHumansModels, GeneticOncogene Proteins, FusionPaired Box Transcription FactorsPAX3 Transcription FactorPAX7 Transcription FactorPhenotypeProtein Structure, TertiaryRhabdomyosarcoma, AlveolarTranscriptional ActivationDNA-Binding ProteinsForkhead Box Protein O1Forkhead Transcription FactorsFOXO1 protein, humanHomeodomain ProteinsOncogene Proteins, FusionPaired Box Transcription FactorsPAX3 protein, humanPax3 protein, mousePAX3 Transcription FactorPAX7 protein, humanPAX7 Transcription FactorTranscription Factors

Identifiers

PMID11607823
OpenAlexW2057793681

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.