ArticleScientific reports2013
HnRNP L and hnRNP LL antagonistically modulate PTB-mediated splicing suppression of CHRNA1 pre-mRNA.
Article in Scientific reports, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 47 citations in OpenAlex.
- Review
- The impact of Hnrnpl deficiency on transcriptional patterns of developing muscle cells.FEBS open bio · 2026Article
- SRSF6 and SRSF1 coordinately enhance the inclusion of humanNAR molecular medicine · 2025Article
- hnRNPs: roles in neurodevelopment and implication for brain disorders.Frontiers in molecular neuroscience · 2024Review
- Review
- Splicing regulation of GFPT1 muscle-specific isoform and its roles in glucose metabolisms and neuromuscular junction.iScience · 2023Article
- Neural Isoforms of Agrin Are Generated by Reduced PTBP1-RNA Interaction Network Spanning the Neuron-Specific Splicing Regions inInternational journal of molecular sciences · 2023Article
- Clinical and Pathologic Features of Congenital Myasthenic Syndromes Caused by 35 Genes-A Comprehensive Review.International journal of molecular sciences · 2023Review
- The HTLV-1 viral oncoproteins Tax and HBZ reprogram the cellular mRNA splicing landscape.PLoS pathogens · 2021Article
- Differential RNA packaging into small extracellular vesicles by neurons and astrocytes.Cell communication and signaling : CCS · 2021Article
- The methyltransferase SETD2 couples transcription and splicing by engaging mRNA processing factors through its SHI domain.Nature communications · 2021Article
- Identification of SRSF10 as a regulator of SMN2 ISS-N1.Human mutation · 2021Article
- Posttranscriptional Gene Regulatory Networks in Chronic Airway Inflammatory Diseases:Frontiers in immunology · 2020Article
- Transcriptome protection by the expanded family of hnRNPs.RNA biology · 2019Review
- Post-Translational Modifications in Polypyrimidine Tract Binding Proteins PTBP1 and PTBP2.Biochemistry · 2018Article
- Multilevel Differential Control of Hormone Gene Expression Programs by hnRNP L and LL in Pituitary Cells.Molecular and cellular biology · 2018Article
- SRSF1 suppresses selection of intron-distal 5' splice site of DOK7 intron 4 to generate functional full-length Dok-7 protein.Scientific reports · 2017Article
- HnRNP-L mediates bladder cancer progression by inhibiting apoptotic signaling and enhancing MAPK signaling pathways.Oncotarget · 2017Article
- Handshakes and Fights: The Regulatory Interplay of RNA-Binding Proteins.Frontiers in molecular biosciences · 2017Review
- Sequence variation between 462 human individuals fine-tunes functional sites of RNA processing.Scientific reports · 2016Article
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 3 countries.
Funding
Abstract
CHRNA1 gene, encoding the muscle nicotinic acetylcholine receptor alpha subunit, harbors an inframe exon P3A. Inclusion of exon P3A disables assembly of the acetylcholine receptor subunits. A single nucleotide mutation in exon P3A identified in congenital myasthenic syndrome causes exclusive inclusion of exon P3A. The mutation gains a de novo binding affinity for a splicing enhancing RNA-binding protein, hnRNP LL, and displaces binding of a splicing suppressing RNA-binding protein, hnRNP L. The hnRNP L binds to another splicing repressor PTB through the proline-rich region and promotes PTB binding to the polypyrimidine tract upstream of exon P3A, whereas hnRNP LL lacking the proline-rich region cannot bind to PTB. Interaction of hnRNP L with PTB inhibits association of U2AF(65) and U1 snRNP with the upstream and downstream of P3A, respectively, which causes a defect in exon P3A definition. HnRNP L and hnRNP LL thus antagonistically modulate PTB-mediated splicing suppression of exon P3A.
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