ArticleThe Journal of biological chemistry2008
The RNA-binding protein CUGBP1 regulates stability of tumor necrosis factor mRNA in muscle cells: implications for myotonic dystrophy.
Article in The Journal of biological chemistry, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 60 papers.
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Who cites it
60 citing papers in PubMed, 88 citations in OpenAlex.
- Therapeutic advances in type 1 myotonic dystrophy complicated with type 2 diabetes mellitus.Frontiers in neurology · 2025Review
- The sustained expression of Cas9 targeting toxic RNAs reverses disease phenotypes in mouse models of myotonic dystrophy type 1.Nature biomedical engineering · 2021Article
- Comprehensive analysis of circRNA expression profiles and circRNA-associated competing endogenous RNA networks in the development of mouse thymus.Journal of cellular and molecular medicine · 2020Article
- Regulation of pro- and anti-atherogenic cytokines.Cytokine · 2019Review
- RNA-binding protein CELF6 is cell cycle regulated and controls cancer cell proliferation by stabilizing p21.Cell death & disease · 2019Article
- HuR counteracts miR-330 to promote STAT3 translation during inflammation-induced muscle wasting.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Training program-induced skeletal muscle adaptations in two men with myotonic dystrophy type 1.BMC research notes · 2019Article
- What is known about the effects of exercise or training to reduce skeletal muscle impairments of patients with myotonic dystrophy type 1? A scoping review.BMC musculoskeletal disorders · 2019Article
- Post-transcriptional regulation of Pabpn1 by the RNA binding protein HuR.Nucleic acids research · 2018Article
- Extra-adrenal glucocorticoids contribute to the postprandial increase of circulating leptin in mice.Journal of cell communication and signaling · 2018Article
- RNA Binding Proteins in Intestinal Epithelial Biology and Colorectal Cancer.Trends in molecular medicine · 2018Review
- Emerging roles of RNA-binding proteins in diabetes and their therapeutic potential in diabetic complications.Wiley interdisciplinary reviews. RNA · 2018Review
- Post-transcriptional regulation of cytokine expression and signaling.Current trends in immunology · 2018Article
- Deregulation of RNA Metabolism in Microsatellite Expansion Diseases.Advances in neurobiology · 2018Review
- Posttranscriptional regulation of intestinal epithelial integrity by noncoding RNAs.Wiley interdisciplinary reviews. RNA · 2017Review
- The CELF1 RNA-Binding Protein Regulates Decay of Signal Recognition Particle mRNAs and Limits Secretion in Mouse Myoblasts.PloS one · 2017Article
- Roles for RNA-binding proteins in development and disease.Brain research · 2016Review
- Article
- Identification of Targets of CUG-BP, Elav-Like Family Member 1 (CELF1) Regulation in Embryonic Heart Muscle.PloS one · 2016Article
- Overexpression of CUGBP1 is associated with the progression of non-small cell lung cancer.Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine · 2015Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Type I myotonic dystrophy (DM1) is caused by a triplet repeat expansion in the 3'-untranslated region (UTR) of the dystrophia myotonia protein kinase (DMPK) gene. Pathogenesis is closely linked with production of a toxic RNA from the mutant allele, which interferes with function of several RNA-binding proteins, including CUGBP1. Here we show that expression of a mutant DMPK 3'-UTR containing 960 CUG repeats is sufficient to increase expression and stability of an mRNA encoding the potent proinflammatory cytokine, tumor necrosis factor (TNF). CUGBP1 specifically recognizes sequences within the TNF 3'-UTR that are dissimilar from its canonical UG-rich binding site. Depletion of CUGBP1 from mouse myoblasts results in increased abundance of TNF mRNA through stabilization of the transcript. Moreover, activation of the protein kinase C pathway by treatment with phorbol ester, which has been shown previously to result in CUGBP1 phosphorylation, also causes TNF mRNA stabilization. Our results suggest that the elevated serum TNF seen in DM1 patients may be derived from muscle where it is induced by expression of toxic DMPK RNA. Importantly, overexpression of this potent cytokine could contribute to the muscle wasting and insulin resistance that are characteristic of this debilitating disease.
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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.