ArticleThe American journal of pathology2011
RNA Foci, CUGBP1, and ZNF9 are the primary targets of the mutant CUG and CCUG repeats expanded in myotonic dystrophies type 1 and type 2.
Article in The American journal of pathology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 38 citations in OpenAlex.
- The role of CNBP in brain atrophy and its targeting in myotonic dystrophy type 2.Human molecular genetics · 2025Article
- Chronic endoplasmic reticulum stress in myotonic dystrophy type 2 promotes autoimmunity via mitochondrial DNA release.Nature communications · 2024Article
- Reducing the Excess Activin Signaling Rescues Muscle Degeneration in Myotonic Dystrophy Type 2Journal of personalized medicine · 2022Article
- Autoimmune Diseases in Patients With Myotonic Dystrophy Type 2.Frontiers in neurology · 2022Article
- Characterisation of Non-Pathogenic Premutation-Range Myotonic Dystrophy Type 2 Alleles.Journal of clinical medicine · 2021Article
- Synaptopathy Mechanisms in ALS Caused by C9orf72 Repeat Expansion.Frontiers in cellular neuroscience · 2021Article
- C9orf72 loss-of-function: a trivial, stand-alone or additive mechanism in C9 ALS/FTD?Acta neuropathologica · 2020Review
- Review
- RNA toxicity in non-coding repeat expansion disorders.The EMBO journal · 2020Review
- Correction of Glycogen Synthase Kinase 3β in Myotonic Dystrophy 1 Reduces the Mutant RNA and Improves Postnatal Survival of DMSXL Mice.Molecular and cellular biology · 2019Article
- rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences.Nature communications · 2018Article
- (CCUG)Disease models & mechanisms · 2017Article
- A G-Rich Motif in the lncRNA Braveheart Interacts with a Zinc-Finger Transcription Factor to Specify the Cardiovascular Lineage.Molecular cell · 2016Article
- Reduction of toxic RNAs in myotonic dystrophies type 1 and type 2 by the RNA helicase p68/DDX5.Proceedings of the National Academy of Sciences of the United States of America · 2015Article
- Molecular mechanisms in DM1 - a focus on foci.Nucleic acids research · 2015Review
- DDX6 regulates sequestered nuclear CUG-expanded DMPK-mRNA in dystrophia myotonica type 1.Nucleic acids research · 2014Article
- Bridging integrator 1 (BIN1) protein expression increases in the Alzheimer's disease brain and correlates with neurofibrillary tangle pathology.Journal of Alzheimer's disease : JAD · 2014Article
- Developing bivalent ligands to target CUG triplet repeats, the causative agent of myotonic dystrophy type 1.Journal of medicinal chemistry · 2013Article
- Molecular mechanisms of muscle atrophy in myotonic dystrophies.The international journal of biochemistry & cell biology · 2013Review
- Antisense oligonucleotides: rising stars in eliminating RNA toxicity in myotonic dystrophy.Human gene therapy · 2013Review
Corrections and comments
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Authors and funding
11 authors at 6 institutions in 3 countries.
Funding
Abstract
Expansions of noncoding CUG and CCUG repeats in myotonic dystrophies type 1 (DM1) and DM2 cause complex molecular pathology, the features of which include accumulation of RNA aggregates and misregulation of the RNA-binding proteins muscleblind-like 1 (MBNL1) and CUG-binding protein 1 (CUGBP1). CCUG repeats also decrease amounts of the nucleic acid binding protein ZNF9. Using tetracycline (Tet)-regulated monoclonal cell models that express CUG and CCUG repeats, we found that low levels of long CUG and CCUG repeats result in nuclear and cytoplasmic RNA aggregation with a simultaneous increase of CUGBP1 and a reduction of ZNF9. Elevation of CUGBP1 and reduction of ZNF9 were also observed before strong aggregation of the mutant CUG/CCUG repeats. Degradation of CUG and CCUG repeats normalizes ZNF9 and CUGBP1 levels. Comparison of short and long CUG and CCUG RNAs showed that great expression of short repeats form foci and alter CUGBP1 and ZNF9; however, long CUG/CCUG repeats misregulate CUGBP1 and ZNF9 much faster than high levels of the short repeats. These data suggest that correction of DM1 and DM2 might be achieved by complete and efficient degradation of CUG and CCUG repeats or by a simultaneous disruption of CUG/CCUG foci and correction of CUGBP1 and ZNF9.
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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.