ArticleMolecular and cellular biology2012
Combinatorial mutagenesis of MBNL1 zinc fingers elucidates distinct classes of regulatory events.
Article in Molecular and cellular biology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 26 citations in OpenAlex.
- Muscleblind-like proteins dimerize by forming disulfide bonds to regulate alternative splicing and pathogenic RNA foci formation.Nucleic acids research · 2026Article
- Muscleblind-like proteins dimerize by forming disulfide bonds to regulate alternative splicing and pathogenic RNA foci formation.bioRxiv : the preprint server for biology · 2026Article
- MBNL proteins in health, disease, and therapeutic applications.Nucleic acids research · 2026Review
- Functions of the Muscleblind-like protein family and their role in disease.Cell communication and signaling : CCS · 2025Review
- Muscleblind-like proteins use modular domains to localize RNAs by riding kinesins and docking to membranes.Nature communications · 2023Article
- Cognate RNA-Binding Modes by the Alternative-Splicing Regulator MBNL1 Inferred from Molecular Dynamics.International journal of molecular sciences · 2022Article
- circMbl functions in cis and in trans to regulate gene expression and physiology in a tissue-specific fashion.Cell reports · 2022Article
- The potential of engineered eukaryotic RNA-binding proteins as molecular tools and therapeutics.Wiley interdisciplinary reviews. RNA · 2020Review
- 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
- Full-length mRNA sequencing uncovers a widespread coupling between transcription initiation and mRNA processing.Genome biology · 2018Article
- Structural Basis for Interaction of the Tandem Zinc Finger Domains of Human Muscleblind with Cognate RNA from Human Cardiac Troponin T.Biochemistry · 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
- Mechanistic determinants of MBNL activity.Nucleic acids research · 2016Article
- Conservation of context-dependent splicing activity in distant Muscleblind homologs.Nucleic acids research · 2016Article
- Dose-Dependent Regulation of Alternative Splicing by MBNL Proteins Reveals Biomarkers for Myotonic Dystrophy.PLoS genetics · 2016Article
- 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
- MBNL proteins and their target RNAs, interaction and splicing regulation.Nucleic acids research · 2014Review
- Dissecting domains necessary for activation and repression of splicing by Muscleblind-like protein 1.BMC molecular biology · 2013Article
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
The RNA binding protein and alternative splicing factor Muscleblind-like 1 (MBNL1) has been a topic of intense study due to its role in myotonic dystrophy (DM) pathogenesis. MBNL1 contains four zinc finger (ZF) RNA binding domains arranged in two pairs. Through combinatorial mutagenesis of the ZF domains, we demonstrate that the pairs of ZFs have differential affinity for RNA and subsequently differential splicing activities. We evaluated splicing and binding activity for six MBNL1-mediated splicing events and found that the splicing activity profiles for the ZF mutants vary among transcripts. Clustering analysis of splicing profiles revealed that two distinct classes of MBNL1 pre-mRNA substrates exist. For some of the RNA transcripts tested, binding and splicing activity of the ZF mutants correlated. However, for some transcripts it appears that MBNL1 exerts robust splicing activity in the absence of RNA binding. We demonstrate that functionally distinct classes of MBNL1-mediated splicing events exist as defined by requirements for ZF-RNA interactions.
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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.