ArticleThe Journal of clinical investigation2016
The nonsense-mediated RNA decay pathway is disrupted in inflammatory myofibroblastic tumors.
Article in The Journal of clinical investigation, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers.
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Who cites it
39 citing papers in PubMed, 61 citations in OpenAlex.
- Bioinspired silk-catechol-enzyme co-assemblies as jigsaw-designed conformation-locking bioactive-biocatalytic platforms.Bioactive materials · 2026Article
- Iron overload suppresses LKB1 and induces IL36G anti-tumor immunity in PDAC metastasis.Science advances · 2026Article
- S100A14 promotes colorectal cancer progression and anti-PD-1 resistance via UPF1-mediated activation of the non-canonical NF-κB signaling.Cell death & disease · 2026Article
- Nonsense-mediated mRNA decay: friend or foe in cancer biology?Cell communication and signaling : CCS · 2026Review
- Nonsense-mediated mRNA decay: a key regulatory system engaged in cancer.Cell communication and signaling : CCS · 2025Review
- Nonsense-Mediated mRNA Decay in Human Health and Diseases: Current Understanding, Regulatory Mechanisms and Future Perspectives.Molecular biotechnology · 2025Review
- High-throughput assay confirmation of a T-cell receptor pre-mRNA fragment as a blood-based inflammatory breast cancer biomarker.medRxiv : the preprint server for health sciences · 2025Article
- Nonsense-Mediated mRNA Decay: Mechanistic Insights and Physiological Significance.Molecular biotechnology · 2024Review
- Inhibition of nonsense-mediated mRNA decay reduces the tumorigenicity of human fibrosarcoma cells.NAR cancer · 2023Article
- Combination therapy of a TRPV2 agonist with a TNF inhibitor achieves sustained suppression of disease severity and reduced joint damage.Clinical and experimental immunology · 2023Article
- Review
- UPF1-From mRNA Degradation to Human Disorders.Cells · 2023Review
- Inflammatory myofibroblastic tumor of the urinary bladder: a case report.The Pan African medical journal · 2023Article
- IL-6/STAT3 signaling in tumor cells restricts the expression of frameshift-derived neoantigens by SMG1 induction.Molecular cancer · 2022Article
- Identification and Validation of UPF1 as a Novel Prognostic Biomarker in Renal Clear Cell Carcinoma.Genes · 2022Article
- A pan-tumor-siRNA aptamer chimera to block nonsense-mediated mRNA decay inflames and suppresses tumor progression.Molecular therapy. Nucleic acids · 2022Article
- Nonsense-mediated RNA decay: an emerging modulator of malignancy.Nature reviews. Cancer · 2022Review
- The edited UPF1 is correlated with elevated asparagine synthetase in pancreatic ductal adenocarcinomas.Molecular biology reports · 2022Article
- Inflammatory Myofibroblastic Tumor of the Right Kidney Mimicking a Locally Advanced Renal Carcinoma: A Case Report.Journal of kidney cancer and VHL · 2022Article
- Glaucocalyxin A Inhibits the Malignancies of Gastric Cancer Cells by Downregulating MDM2 and RNF6Frontiers in oncology · 2022Article
Corrections and comments
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Authors and funding
14 authors at 4 institutions in 2 countries.
Funding
Abstract
Inflammatory myofibroblastic tumors (IMTs) are characterized by myofibroblast proliferation and an inflammatory cell infiltrate. Little is known about the molecular pathways that precipitate IMT formation. Here, we report the identification of somatic mutations in UPF1, a gene that encodes an essential component of the nonsense-mediated RNA decay (NMD) pathway, in 13 of 15 pulmonary IMT samples. The majority of mutations occurred in a specific region of UPF1 and triggered UPF1 alternative splicing. Several mRNA targets of the NMD pathway were upregulated in IMT samples, indicating that the UPF1 mutations led to reduced NMD magnitude. These upregulated NMD targets included NIK mRNA, which encodes a potent activator of NF-κB. In human lung cells, UPF1 depletion increased expression of chemokine-encoding genes in a NIK-dependent manner. Elevated chemokines and IgE class switching events were observed in IMT samples, consistent with NIK upregulation in these tumors. Together, these results support a model in which UPF1 mutations downregulate NMD, leading to NIK-dependent NF-κB induction, which contributes to the immune infiltration that is characteristic of IMTs. The molecular link between the NMD pathway and IMTs has implications for the diagnosis and treatment of these tumors.
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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.