Nusinersen ameliorates motor function and prevents motoneuron Cajal body disassembly and abnormal poly(A) RNA distribution in a SMA mouse model
Published 2020 View Full Article
- Home
- Publications
- Publication Search
- Publication Details
Title
Nusinersen ameliorates motor function and prevents motoneuron Cajal body disassembly and abnormal poly(A) RNA distribution in a SMA mouse model
Authors
Keywords
-
Journal
Scientific Reports
Volume 10, Issue 1, Pages -
Publisher
Springer Science and Business Media LLC
Online
2020-07-01
DOI
10.1038/s41598-020-67569-3
References
Ask authors/readers for more resources
Related references
Note: Only part of the references are listed.- Antisense Oligonucleotide Therapies for Neurodegenerative Diseases
- (2019) C. Frank Bennett et al. Annual Review of Neuroscience
- The role of survival motor neuron protein (SMN) in protein homeostasis
- (2018) Helena Chaytow et al. CELLULAR AND MOLECULAR LIFE SCIENCES
- Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA
- (2018) J. Oriol Narcís et al. Scientific Reports
- The clinical landscape for SMA in a new therapeutic era
- (2017) K Talbot et al. GENE THERAPY
- Cellular bases of the RNA metabolism dysfunction in motor neurons of a murine model of spinal muscular atrophy: Role of Cajal bodies and the nucleolus
- (2017) Olga Tapia et al. NEUROBIOLOGY OF DISEASE
- SMN deficiency in severe models of spinal muscular atrophy causes widespread intron retention and DNA damage
- (2017) Mohini Jangi et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- In Vivo Translatome Profiling in Spinal Muscular Atrophy Reveals a Role for SMN Protein in Ribosome Biology
- (2017) Paola Bernabò et al. Cell Reports
- RNA-sequencing of a mouse-model of spinal muscular atrophy reveals tissue-wide changes in splicing of U12-dependent introns
- (2016) Thomas Koed Doktor et al. NUCLEIC ACIDS RESEARCH
- The Cajal body and the nucleolus: “In a relationship” or “It's complicated”?
- (2016) Laura Trinkle-Mulcahy et al. RNA Biology
- Specific genomic cues regulate Cajal body assembly
- (2016) Iain A. Sawyer et al. RNA Biology
- Cajal bodies in neurons
- (2016) Miguel Lafarga et al. RNA Biology
- Cajal bodies are linked to genome conformation
- (2016) Qiuyan Wang et al. Nature Communications
- Skeletal muscle fiber type: using insights from muscle developmental biology to dissect targets for susceptibility and resistance to muscle disease
- (2016) Jared Talbot et al. Wiley Interdisciplinary Reviews-Developmental Biology
- A Battery of Motor Tests in a Neonatal Mouse Model of Cerebral Palsy
- (2016) Danielle N. Feather-Schussler et al. Jove-Journal of Visualized Experiments
- Motor neuron cell-nonautonomous rescue of spinal muscular atrophy phenotypes in mild and severe transgenic mouse models
- (2015) Yimin Hua et al. GENES & DEVELOPMENT
- SART3-Dependent Accumulation of Incomplete Spliceosomal snRNPs in Cajal Bodies
- (2015) Ivan Novotný et al. Cell Reports
- Mechanisms Involved in Spinal Cord Central Synapse Loss in a Mouse Model of Spinal Muscular Atrophy
- (2014) Olga Tarabal et al. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
- The Coilin Interactome Identifies Hundreds of Small Noncoding RNAs that Traffic through Cajal Bodies
- (2014) Martin Machyna et al. MOLECULAR CELL
- A day in the life of the spliceosome
- (2014) A. Gregory Matera et al. NATURE REVIEWS MOLECULAR CELL BIOLOGY
- Inhibition of U4 snRNA in Human Cells Causes the Stable Retention of Polyadenylated Pre-mRNA in the Nucleus
- (2014) Anne Hett et al. PLoS One
- Brain pathology in myotonic dystrophy: when tauopathy meets spliceopathy and RNAopathy
- (2014) Marie-Laure Caillet-Boudin et al. Frontiers in Molecular Neuroscience
- Proteasome inhibition induces DNA damage and reorganizes nuclear architecture and protein synthesis machinery in sensory ganglion neurons
- (2013) Ana Palanca et al. CELLULAR AND MOLECULAR LIFE SCIENCES
- Motor neurone disease: a practical update on diagnosis and management
- (2013) P. J. Shaw et al. CLINICAL MEDICINE
- Chondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophy
- (2013) James N. Sleigh et al. HUMAN MOLECULAR GENETICS
- An SMN-Dependent U12 Splicing Event Essential for Motor Circuit Function
- (2012) Francesco Lotti et al. CELL
- TSUNAMI: an antisense method to phenocopy splicing-associated diseases in animals
- (2012) K. Sahashi et al. GENES & DEVELOPMENT
- Reorganization of Cajal bodies and nucleolar targeting of coilin in motor neurons of type I spinal muscular atrophy
- (2012) Olga Tapia et al. HISTOCHEMISTRY AND CELL BIOLOGY
- Chondrolectin Mediates Growth Cone Interactions of Motor Axons with an Intermediate Target
- (2012) Z. Zhong et al. JOURNAL OF NEUROSCIENCE
- RNA therapeutics: beyond RNA interference and antisense oligonucleotides
- (2012) Ryszard Kole et al. NATURE REVIEWS DRUG DISCOVERY
- Spinal muscular atrophy: going beyond the motor neuron
- (2012) Gillian Hamilton et al. TRENDS IN MOLECULAR MEDICINE
- Cajal bodies: where form meets function
- (2012) Martin Machyna et al. Wiley Interdisciplinary Reviews-RNA
- A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouse
- (2011) Paul N. Porensky et al. HUMAN MOLECULAR GENETICS
- Purkinje Cell Degeneration inpcdMice Reveals Large Scale Chromatin Reorganization and Gene Silencing Linked to Defective DNA Repair
- (2011) Fernando C. Baltanás et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Antisense Oligonucleotides Delivered to the Mouse CNS Ameliorate Symptoms of Severe Spinal Muscular Atrophy
- (2011) M. A. Passini et al. Science Translational Medicine
- Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model
- (2010) Y. Hua et al. GENES & DEVELOPMENT
- A degron created by SMN2 exon 7 skipping is a principal contributor to spinal muscular atrophy severity
- (2010) S. Cho et al. GENES & DEVELOPMENT
- Tropomodulin isoforms regulate thin filament pointed-end capping and skeletal muscle physiology
- (2010) David S. Gokhin et al. JOURNAL OF CELL BIOLOGY
- In vivo kinetics of U4/U6·U5 tri-snRNP formation in Cajal bodies
- (2010) Ivan Novotný et al. MOLECULAR BIOLOGY OF THE CELL
- Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?
- (2009) Arthur H. M. Burghes et al. NATURE REVIEWS NEUROSCIENCE
- Bortezomib Induces the Formation of Nuclear poly(A) RNA Granules Enriched in Sam68 and PABPN1 in Sensory Ganglia Neurons
- (2009) Iñigo Casafont et al. NEUROTOXICITY RESEARCH
- Alternative Splicing Events Are a Late Feature of Pathology in a Mouse Model of Spinal Muscular Atrophy
- (2009) Dirk Bäumer et al. PLoS Genetics
- Antisense Masking of an hnRNP A1/A2 Intronic Splicing Silencer Corrects SMN2 Splicing in Transgenic Mice
- (2008) Yimin Hua et al. AMERICAN JOURNAL OF HUMAN GENETICS
- SMN Deficiency Causes Tissue-Specific Perturbations in the Repertoire of snRNAs and Widespread Defects in Splicing
- (2008) Zhenxi Zhang et al. CELL
Find the ideal target journal for your manuscript
Explore over 38,000 international journals covering a vast array of academic fields.
SearchBecome a Peeref-certified reviewer
The Peeref Institute provides free reviewer training that teaches the core competencies of the academic peer review process.
Get Started