Cargando…

Splicing changes in SMA mouse motoneurons and SMN-depleted neuroblastoma cells: Evidence for involvement of splicing regulatory proteins

Spinal Muscular Atrophy (SMA) is caused by deletions or mutations in the Survival Motor Neuron 1 (SMN1) gene. The second gene copy, SMN2, produces some, but not enough, functional SMN protein. SMN is essential to assemble small nuclear ribonucleoproteins (snRNPs) that form the spliceosome. However,...

Descripción completa

Detalles Bibliográficos
Autores principales:	Huo, Qing, Kayikci, Melis, Odermatt, Philipp, Meyer, Kathrin, Michels, Olivia, Saxena, Smita, Ule, Jernej, Schümperli, Daniel
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Taylor & Francis 2015
Materias:	Research Papers
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4601534/ https://www.ncbi.nlm.nih.gov/pubmed/25692239 http://dx.doi.org/10.1080/15476286.2014.996494

Ejemplares similares

Somatic Therapy of a Mouse SMA Model with a U7 snRNA Gene Correcting SMN2 Splicing
por: Odermatt, Philipp, et al.
Publicado: (2016)

Pathological impact of SMN2 mis-splicing in adult SMA mice
por: Sahashi, Kentaro, et al.
Publicado: (2013)

iCLIP Predicts the Dual Splicing Effects of TIA-RNA Interactions
por: Wang, Zhen, et al.
Publicado: (2010)

Understanding splicing regulation through RNA splicing maps
por: Witten, Joshua T., et al.
Publicado: (2011)

The alternative splicing program of differentiated smooth muscle cells involves concerted non-productive splicing of post-transcriptional regulators
por: Llorian, Miriam, et al.
Publicado: (2016)

Development of 2′-O-Methyl and LNA Antisense Oligonucleotides for SMN2 Splicing Correction in SMA Cells
por: Maretina, Marianna, et al.
Publicado: (2023)

iCLIP reveals the function of hnRNP particles in splicing at individual nucleotide resolution
por: König, Julian, et al.
Publicado: (2010)

Altered mRNA Splicing in SMN-Depleted Motor Neuron-Like Cells
por: Custer, Sara K., et al.
Publicado: (2016)

Nuclear matrix protein Matrin3 regulates alternative splicing and forms overlapping regulatory networks with PTB
por: Coelho, Miguel B, et al.
Publicado: (2015)

LNA/DNA mixmer-based antisense oligonucleotides correct alternative splicing of the SMN2 gene and restore SMN protein expression in type 1 SMA fibroblasts
por: Touznik, Aleksander, et al.
Publicado: (2017)

Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice
por: Mirra, Alessia, et al.
Publicado: (2017)

Smn-Deficiency Increases the Intrinsic Excitability of Motoneurons
por: Arumugam, Saravanan, et al.
Publicado: (2017)

Publisher Correction: Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice
por: Mirra, Alessia, et al.
Publicado: (2018)

MRI correlates of motoneuron loss in SMA
por: Sprenger-Svačina, Alina, et al.
Publicado: (2022)

Alternative splicing of a cryptic exon embedded in intron 6 of SMN1 and SMN2
por: Yoshimoto, Satomi, et al.
Publicado: (2016)

Functional characterization of SMN evolution in mouse models of SMA
por: Osman, Erkan Y., et al.
Publicado: (2019)

Evolution of Nova-Dependent Splicing Regulation in the Brain
por: Jelen, Nejc, et al.
Publicado: (2007)

Characterising the RNA targets and position-dependent splicing regulation by TDP-43; implications for neurodegenerative diseases
por: Tollervey, James R., et al.
Publicado: (2011)

The Need for SMN-Independent Treatments of Spinal Muscular Atrophy (SMA) to Complement SMN-Enhancing Drugs
por: Hensel, Niko, et al.
Publicado: (2020)

Antisense Oligonucleotide Induction of the hnRNPA1b Isoform Affects Pre-mRNA Splicing of SMN2 in SMA Type I Fibroblasts
por: Toosaranont, Jarichad, et al.
Publicado: (2022)

Drug screening with human SMN2 reporter identifies SMN protein stabilizers to correct SMA pathology
por: Wang, Yiran, et al.
Publicado: (2019)

SMA-linked SMN mutants prevent phase separation properties and SMN interactions with FMRP family members
por: Binda, Olivier, et al.
Publicado: (2022)

How do SMA-linked mutations of SMN1 lead to structural/functional deficiency of the SMA protein?
por: Li, Wei
Publicado: (2017)

NOVA1 promotes SMN2 exon 7 splicing by binding the UCAC motif and increases SMN protein expression
por: Du, Li-Li, et al.
Publicado: (2022)

Possible implication of undescribed SMN1-SMN2 genotype in chronic EMG-pattern of SMA with transitory acute denervation
por: Vitello, Girolamo A., et al.
Publicado: (2020)

Splicing Defects of the Profilin Gene Alter Actin Dynamics in an S. pombe SMN Mutant
por: Antoine, Marie, et al.
Publicado: (2019)

Psip1/Ledgf p52 Binds Methylated Histone H3K36 and Splicing Factors and Contributes to the Regulation of Alternative Splicing
por: Pradeepa, Madapura M., et al.
Publicado: (2012)

Transcriptional enhancement of Smn levels in motoneurons is crucial for proper axon morphology in zebrafish
por: Spiró, Zoltán, et al.
Publicado: (2016)

RNAmotifs: prediction of multivalent RNA motifs that control alternative splicing
por: Cereda, Matteo, et al.
Publicado: (2014)

The Carrier Frequency of Two SMN1 Genes in Parents of Symptomatic Children with SMA and the Significance of SMN1 Exon 8 in Carriers
por: Davidson, Joanne E, et al.
Publicado: (2023)

Absence of an Intron Splicing Silencer in Porcine Smn1 Intron 7 Confers Immunity to the Exon Skipping Mutation in Human SMN2
por: Doktor, Thomas Koed, et al.
Publicado: (2014)

Cervical Spinal Cord Atrophy Profile in Adult SMN1-Linked SMA
por: El Mendili, Mohamed-Mounir, et al.
Publicado: (2016)

A comprehensive overview of SMN and NAIP copy numbers in Iranian SMA patients
por: Savad, Shahram, et al.
Publicado: (2023)

The Neuronal Splicing Factor Nova Co-Localizes with Target RNAs in the Dendrite
por: Racca, Claudia, et al.
Publicado: (2010)

Development of a Single Vector System that Enhances Trans-Splicing of SMN2 Transcripts
por: Coady, Tristan H., et al.
Publicado: (2008)

SAM68 is a physiological regulator of SMN2 splicing in spinal muscular atrophy
por: Pagliarini, Vittoria, et al.
Publicado: (2015)

Regulation of alternative splicing by the circadian clock and food related cues
por: McGlincy, Nicholas J, et al.
Publicado: (2012)

Modeling Drosophila motoneurons to examine the functional effect of Na channel splice variants
por: Günay, Cengiz, et al.
Publicado: (2010)

A homozygous double mutation in SMN1: a complicated genetic diagnosis of SMA
por: Kirwin, Susan M, et al.
Publicado: (2013)

Correction: Cervical Spinal Cord Atrophy Profile in Adult SMN1-Linked SMA
por: El Mendili, Mohamed-Mounir, et al.
Publicado: (2016)

Cannot write session to /tmp/vufind_sessions/sess_sq0r1edl68gr2tshf1i6iqnm2h