ArticleBMC molecular biology2011
The four Zn fingers of MBNL1 provide a flexible platform for recognition of its RNA binding elements.
Article in BMC molecular biology, 2011. 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, 41 citations in OpenAlex.
- MBNL proteins in health, disease, and therapeutic applications.Nucleic acids research · 2026Review
- Targeting Expanded CUG and CTG Repeats as a Therapeutic Approach for Myotonic Dystrophy Type 1 (DM1).ChemMedChem · 2026Review
- Article
- Transcription FactorJournal of fungi (Basel, Switzerland) · 2023Article
- Transcriptome-wide identification of single-stranded RNA binding proteins.Chemical science · 2023Article
- Review
- An Overview of Circular RNAs and Their Implications in Myotonic Dystrophy.International journal of molecular sciences · 2019Review
- Short Tandem Repeat Expansions and RNA-Mediated Pathogenesis in Myotonic Dystrophy.International journal of molecular sciences · 2019Review
- Expanded CUG repeats inRNA (New York, N.Y.) · 2019Article
- MBNL splicing activity depends on RNA binding site structural context.Nucleic acids research · 2018Article
- An engineered RNA binding protein with improved splicing regulation.Nucleic acids research · 2018Article
- Structural Basis for Interaction of the Tandem Zinc Finger Domains of Human Muscleblind with Cognate RNA from Human Cardiac Troponin T.Biochemistry · 2017Article
- Pseudouridine Modification Inhibits Muscleblind-like 1 (MBNL1) Binding to CCUG Repeats and Minimally Structured RNA through Reduced RNA Flexibility.The Journal of biological chemistry · 2017Article
- Autoregulation of MBNL1 function by exon 1 exclusion from MBNL1 transcript.Nucleic acids research · 2017Article
- Structural Characteristics of Simple RNA Repeats Associated with Disease and their Deleterious Protein Interactions.Frontiers in cellular neuroscience · 2017Review
- Identification of variants in MBNL1 in patients with a myotonic dystrophy-like phenotype.European journal of human genetics : EJHG · 2016Article
- Computational investigation of RNA CUG repeats responsible for myotonic dystrophy 1.Journal of chemical theory and computation · 2015Article
- Molecular mechanisms in DM1 - a focus on foci.Nucleic acids research · 2015Review
- Myocardial ischemic preconditioning upregulated protein 1(Mipu1):zinc finger protein 667 - a multifunctional KRAB/C2H2 zinc finger protein.Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologica · 2015Article
- Modifications to toxic CUG RNAs induce structural stability, rescue mis-splicing in a myotonic dystrophy cell model and reduce toxicity in a myotonic dystrophy zebrafish model.Nucleic acids research · 2014Article
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Authors and funding
6 authors at 3 institutions in 1 country.
Funding
Abstract
backgroundMuscleblind-like 1 (MBNL1) is an alternative splicing factor containing four CCCH Zinc fingers (ZnFs). The sequestration of MBNL1 by expanded CUG and CCUG repeats is a major component in causing myotonic dystrophy. In addition to binding the structured expanded CUG and CCUG repeats; previous results suggested that MBNL1 binds single-stranded RNAs containing GC dinucleotides.
resultsWe performed a systematic analysis of MBNL1 binding to single-stranded RNAs. These studies revealed that a single GC dinucleotide in poly-uridine is sufficient for MBNL1 binding and that a second GC dinucleotide confers higher affinity MBNL1 binding. However additional GC dinucleotides do not enhance RNA binding. We also showed that the RNA sequences adjacent to the GC dinucleotides play an important role in MBNL1 binding with the following preference: uridines >cytidines >adenosines >guanosines. For high affinity binding by MBNL1, the distance between the two GC dinucleotides can vary from 1 to 17 nucleotides.
conclusionsThese results suggest that MBNL1 is highly flexible and able to adopt different conformations to recognize RNAs with varying sequence configurations. Although MBNL1 contains four ZnFs, only two ZnF - GC dinucleotide interactions are necessary for high affinity binding.
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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.