Cargando…

Altered levels of the splicing factor muscleblind modifies cerebral cortical function in mouse models of myotonic dystrophy

Myotonic dystrophy (DM) is a progressive, multisystem disorder affecting skeletal muscle, heart, and central nervous system. In both DM1 and DM2, microsatellite expansions of CUG and CCUG RNA repeats, respectively, accumulate and disrupt functions of alternative splicing factors, including musclebli...

Descripción completa

Detalles Bibliográficos
Autores principales:	Chen, Gang, Carter, Russell E., Cleary, John D., Reid, Tammy S., Ranum, Laura P., Swanson, Maurice S., Ebner, Timothy J.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	2018
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5859959/ https://www.ncbi.nlm.nih.gov/pubmed/29331264 http://dx.doi.org/10.1016/j.nbd.2018.01.003

Ejemplares similares

Compound loss of muscleblind-like function in myotonic dystrophy
por: Lee, Kuang-Yung, et al.
Publicado: (2013)

Muscleblind-Like 1 Knockout Mice Reveal Novel Splicing Defects in the Myotonic Dystrophy Brain
por: Suenaga, Koichi, et al.
Publicado: (2012)

Zebrafish deficient for Muscleblind-like 2 exhibit features of myotonic dystrophy
por: Machuca-Tzili, Laura E., et al.
Publicado: (2011)

Muscleblind, BSF and TBPH are mislocalized in the muscle sarcomere of a Drosophila myotonic dystrophy model
por: Llamusi, Beatriz, et al.
Publicado: (2013)

Muscleblind-like1 Undergoes Ectopic Relocation in the Nuclei of Skeletal Muscles in Myotonic Dystrophy and Sarcopenia
por: Malatesta, M., et al.
Publicado: (2013)

Derepressing muscleblind expression by miRNA sponges ameliorates myotonic dystrophy-like phenotypes in Drosophila
por: Cerro-Herreros, Estefania, et al.
Publicado: (2016)

Muscleblind-like 3 deficit results in a spectrum of age-associated pathologies observed in myotonic dystrophy
por: Choi, Jongkyu, et al.
Publicado: (2016)

Altered Behavioral Responses Show GABA Sensitivity in Muscleblind-Like 2-Deficient Mice: Implications for CNS Symptoms in Myotonic Dystrophy
por: Edokpolor, Kamyra S., et al.
Publicado: (2022)

Optical Mapping Approaches on Muscleblind‐Like Compound Knockout Mice for Understanding Mechanistic Insights Into Ventricular Arrhythmias in Myotonic Dystrophy
por: Chou, Chung‐Chuan, et al.
Publicado: (2017)

Muscleblind-like 1 interacts with RNA hairpins in splicing target and pathogenic RNAs
por: Yuan, Yuan, et al.
Publicado: (2007)

Muscleblind1, but Not Dmpk or Six5, Contributes to a Complex Phenotype of Muscular and Motivational Deficits in Mouse Models of Myotonic Dystrophy
por: Matynia, Anna, et al.
Publicado: (2010)

Deterministic splicing of Dscam2 is regulated by Muscleblind
por: Li, Joshua Shing Shun, et al.
Publicado: (2019)

miR-23b and miR-218 silencing increase Muscleblind-like expression and alleviate myotonic dystrophy phenotypes in mammalian models
por: Cerro-Herreros, Estefania, et al.
Publicado: (2018)

Autistic traits in myotonic dystrophy type 1 due to MBNL inhibition and RNA mis-splicing
por: Sznajder, Lukasz, et al.
Publicado: (2023)

Drosophila Muscleblind Is Involved in troponin T Alternative Splicing and Apoptosis
por: Vicente-Crespo, Marta, et al.
Publicado: (2008)

Short Tandem Repeat Expansions and RNA-Mediated Pathogenesis in Myotonic Dystrophy
por: Sznajder, Łukasz J., et al.
Publicado: (2019)

Cell-type-specific dysregulation of RNA alternative splicing in short tandem repeat mouse knockin models of myotonic dystrophy
por: Nutter, Curtis A., et al.
Publicado: (2019)

Conservation of context-dependent splicing activity in distant Muscleblind homologs
por: Oddo, Julia C., et al.
Publicado: (2016)

Abnormal splicing switch of DMD's penultimate exon compromises muscle fibre maintenance in myotonic dystrophy
por: Rau, Frédérique, et al.
Publicado: (2015)

Myotonic dystrophy
por: Patterson, VH
Publicado: (1990)

Muscle Chloride Channel Dysfunction in Two Mouse Models of Myotonic Dystrophy
por: Lueck, John D., et al.
Publicado: (2007)

Staufen1s role as a splicing factor and a disease modifier in Myotonic Dystrophy Type I
por: Bondy-Chorney, Emma, et al.
Publicado: (2016)

An Overview of Alternative Splicing Defects Implicated in Myotonic Dystrophy Type I
por: López-Martínez, Andrea, et al.
Publicado: (2020)

Muscleblind-like 1 is required for normal heart valve development in vivo
por: Coram, Ryan J., et al.
Publicado: (2015)

Molecular characterization of myotonic dystrophy fibroblast cell lines for use in small molecule screening
por: Jenquin, Jana R., et al.
Publicado: (2022)

Cancer and Myotonic Dystrophy
por: D’Ambrosio, Eleonora S., et al.
Publicado: (2023)

Disrupted prenatal RNA processing and myogenesis in congenital myotonic dystrophy
por: Thomas, James D., et al.
Publicado: (2017)

Dissecting domains necessary for activation and repression of splicing by muscleblind-like protein 1
por: Edge, Christopher, et al.
Publicado: (2013)

Mitigating RNA Toxicity in Myotonic Dystrophy using Small Molecules
por: Reddy, Kaalak, et al.
Publicado: (2019)

Targeted splice sequencing reveals RNA toxicity and therapeutic response in myotonic dystrophy
por: Tanner, Matthew K, et al.
Publicado: (2021)

HNRNPA1-induced spliceopathy in a transgenic mouse model of myotonic dystrophy
por: Li, Moyi, et al.
Publicado: (2020)

Splicing misregulation of SCN5A contributes to cardiac-conduction delay and heart arrhythmia in myotonic dystrophy
por: Freyermuth, Fernande, et al.
Publicado: (2016)

Transcriptome alterations in myotonic dystrophy frontal cortex
por: Otero, Brittney A., et al.
Publicado: (2021)

Muscleblind-Like 1 and Muscleblind-Like 3 Depletion Synergistically Enhances Myotonia by Altering Clc-1 RNA Translation
por: Choi, Jongkyu, et al.
Publicado: (2015)

Loss of muscleblind-like 1 results in cardiac pathology and persistence of embryonic splice isoforms
por: Dixon, Donald M., et al.
Publicado: (2015)

Desflurane anaesthesia in myotonic dystrophy
por: Gandhi, Ranju, et al.
Publicado: (2011)

Myotonic Dystrophy: An Anaesthetic Dilemma
por: Gupta, N, et al.
Publicado: (2009)

Cardiac involvement in myotonic dystrophy
por: Khalighi, Koroush, et al.
Publicado: (2015)

Steakhouse Syndrome in Myotonic Dystrophy
por: Ogasawara, Nobuhiko, et al.
Publicado: (2017)

Cutaneous findings in myotonic dystrophy
por: Kong, Ha Eun, et al.
Publicado: (2022)

Cannot write session to /tmp/vufind_sessions/sess_1soogorv196f61d8p4hf0elubn