ArticleThe Journal of cell biology2002
Mutant DMPK 3'-UTR transcripts disrupt C2C12 myogenic differentiation by compromising MyoD.
Article in The Journal of cell biology, 2002. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 55 citations in OpenAlex.
- Structural and Dynamical Properties of Nucleic Acid Hairpins Implicated in Trinucleotide Repeat Expansion Diseases.Biomolecules · 2024Review
- Profiling muscle transcriptome in mice exposed to microgravity using gene set enrichment analysis.NPJ microgravity · 2024Article
- Pluripotent Stem Cells in Disease Modeling and Drug Discovery for Myotonic Dystrophy Type 1.Cells · 2023Review
- Modeling muscle regeneration in RNA toxicity mice.Human molecular genetics · 2021Article
- Inhibition ofFrontiers in cell and developmental biology · 2021Article
- miR-322/-503 rescues myoblast defects in myotonic dystrophy type 1 cell model by targeting CUG repeats.Cell death & disease · 2020Article
- Dosage effect of multiple genes accounts for multisystem disorder of myotonic dystrophy type 1.Cell research · 2020Article
- Correction of RNA-Binding Protein CUGBP1 and GSK3β Signaling as Therapeutic Approach for Congenital and Adult Myotonic Dystrophy Type 1.International journal of molecular sciences · 2019Review
- Recovery in the Myogenic Program of Congenital Myotonic Dystrophy Myoblasts after Excision of the Expanded (CTG)International journal of molecular sciences · 2019Article
- MBNL1 overexpression is not sufficient to rescue the phenotypes in a mouse model of RNA toxicity.Human molecular genetics · 2019Article
- Activation of the interferon type I response rather than autophagy contributes to myogenesis inhibition in congenital DM1 myoblasts.Cell death & disease · 2018Article
- Immortalized human myotonic dystrophy muscle cell lines to assess therapeutic compounds.Disease models & mechanisms · 2017Article
- Functional KCa1.1 channels are crucial for regulating the proliferation, migration and differentiation of human primary skeletal myoblasts.Cell death & disease · 2016Article
- A novel myogenic function residing in the 5' non-coding region of Insulin receptor substrate-1 (Irs-1) transcript.BMC cell biology · 2015Article
- Altered nuclear structure in myotonic dystrophy type 1-derived fibroblasts.Molecular biology reports · 2015Article
- NKX2-5, a modifier of skeletal muscle pathology due to RNA toxicity.Human molecular genetics · 2015Article
- The RNA-binding protein Staufen1 impairs myogenic differentiation via a c-myc-dependent mechanism.Molecular biology of the cell · 2014Article
- Myoblasts generated by lentiviral mediated MyoD transduction of myotonic dystrophy type 1 (DM1) fibroblasts can be used for assays of therapeutic molecules.BMC research notes · 2011Article
- Cellular toxicity of expanded RNA repeats: focus on RNA foci.Human molecular genetics · 2011Review
- CLOCK and BMAL1 regulate MyoD and are necessary for maintenance of skeletal muscle phenotype and function.Proceedings of the National Academy of Sciences of the United States of America · 2010Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
Myotonic dystrophy (DM) is caused by two similar noncoding repeat expansion mutations (DM1 and DM2). It is thought that both mutations produce pathogenic RNA molecules that accumulate in nuclear foci. The DM1 mutation is a CTG expansion in the 3' untranslated region (3'-UTR) of dystrophia myotonica protein kinase (DMPK). In a cell culture model, mutant transcripts containing a (CUG)200 DMPK 3'-UTR disrupt C2C12 myoblast differentiation; a phenotype similar to what is observed in myoblast cultures derived from DM1 patient muscle. Here, we have used our cell culture model to investigate how the mutant 3'-UTR RNA disrupts differentiation. We show that MyoD protein levels are compromised in cells that express mutant DMPK 3'-UTR transcripts. MyoD, a transcription factor required for the differentiation of myoblasts during muscle regeneration, activates differentiation-specific genes by binding E-boxes. MyoD levels are significantly reduced in myoblasts expressing the mutant 3'-UTR RNA within the first 6 h under differentiation conditions. This reduction correlates with blunted E-box-mediated gene expression at time points that are critical for initiating differentiation. Importantly, restoring MyoD levels rescues the differentiation defect. We conclude that mutant DMPK 3'-UTR transcripts disrupt myoblast differentiation by reducing MyoD levels below a threshold required to activate the differentiation program.
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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.