ArticleGenes & development2003
The transcriptional activator PAX3-FKHR rescues the defects of Pax3 mutant mice but induces a myogenic gain-of-function phenotype with ligand-independent activation of Met signaling in vivo.
Article in Genes & development, 2003. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 69 papers.
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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.
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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.
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Who cites it
69 citing papers in PubMed, 147 citations in OpenAlex.
- PAX3: A Driver of Normal Development and Disease.Biomolecules · 2026Review
- Regulation and function of Pax3 in muscle stem cell heterogeneity and stress response.Journal of muscle research and cell motility · 2025Review
- Dual transcriptional activities of PAX3 and PAX7 spatially encode spinal cell fates through distinct gene networks.PLoS biology · 2025Article
- Article
- Pitx2 Differentially Regulates the Distinct Phases of Myogenic Program and Delineates Satellite Cell Lineages During Muscle Development.Frontiers in cell and developmental biology · 2022Article
- The hepatocyte growth factor/mesenchymal epithelial transition factor axis in high-risk pediatric solid tumors and the anti-tumor activity of targeted therapeutic agents.Frontiers in pediatrics · 2022Review
- Master regulators of skeletal muscle lineage development and pluripotent stem cells differentiation.Cell regeneration (London, England) · 2021Review
- Systemic cell therapy for muscular dystrophies : The ultimate transplantable muscle progenitor cell and current challenges for clinical efficacy.Stem cell reviews and reports · 2021Review
- M-Cadherin Is a PAX3 Target During Myotome Patterning.Frontiers in cell and developmental biology · 2021Article
- Article
- Fgf10/Fgfr2b Signaling in Mammary Gland Development, Homeostasis, and Cancer.Frontiers in cell and developmental biology · 2020Review
- Review
- PAX3 Confers Functional Heterogeneity in Skeletal Muscle Stem Cell Responses to Environmental Stress.Cell stem cell · 2019Article
- Axial and limb muscle development: dialogue with the neighbourhood.Cellular and molecular life sciences : CMLS · 2016Review
- Intergenically Spliced Chimeric RNAs in Cancer.Trends in cancer · 2016Review
- TBX2 represses PTEN in rhabdomyosarcoma and skeletal muscle.Oncogene · 2016Article
- Article
- Endothelial cell specification in the somite is compromised in Pax3-positive progenitors of Foxc1/2 conditional mutants, with loss of forelimb myogenesis.Development (Cambridge, England) · 2016Article
- Differentiation of pluripotent stem cells to muscle fiber to model Duchenne muscular dystrophy.Nature biotechnology · 2015Article
- Probing for a deeper understanding of rhabdomyosarcoma: insights from complementary model systems.Nature reviews. Cancer · 2015Review
9 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors at 4 institutions in 3 countries.
Funding
Abstract
Pax3 is a key transcription factor implicated in development and human disease. To dissect the role of Pax3 in myogenesis and establish whether it is a repressor or activator, we generated loss- and gain-of-function alleles by targeting an nLacZ reporter and a sequence encoding the oncogenic fusion protein PAX3-FKHR into the Pax3 locus. Rescue of the Pax3 mutant phenotypes by PAX3-FKHR suggests that Pax3 acts as a transcriptional activator during embryogenesis. This is confirmed by a Pax reporter mouse. However, mice expressing PAX3-FKHR display developmental defects, including ectopic delamination and inappropriate migration of muscle precursor cells. These events result from overexpression of c-met, leading to constitutive activation of Met signaling, despite the absence of the ligand SF/HGF. Haploinsufficiency of c-met rescues this phenotype, confirming the direct genetic link with Pax3. The gain-of-function phenotype is also characterized by overactivation of MyoD. The consequences of PAX3-FKHR myogenic activity in the limbs and cervical and thoracic regions point to differential regulation of muscle growth and patterning. This gain-of-function allele provides a new approach to the molecular and cellular analysis of the role of Pax3 and of its target genes in vivo.
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Registered trials
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.