Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy. - Wikidata - wikimedia - TriplyDB

Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy.

Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy.

http://www.wikidata.org/entity/Q42548444

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Restoration of full-length SMN promoted by adenoviral vectors expressing RNA antisense oligonucleotides embedded in U7 snRNAs Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy Survival motor neuron protein regulates stem cell division, proliferation, and differentiation in Drosophila Gemin8 is required for the architecture and function of the survival motor neuron complex Ongoing U snRNP biogenesis is required for the integrity of Cajal bodies Two distinct arginine methyltransferases are required for biogenesis of Sm-class ribonucleoproteins Gemin proteins are required for efficient assembly of Sm-class ribonucleoproteins.The survival motor neuron protein forms soluble glycine zipper oligomers The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy Intronic miR-26b controls neuronal differentiation by repressing its host transcript, ctdsp2 A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice Gemin5 Binds to the Survival Motor Neuron mRNA to Regulate SMN Expression In vitro and in cellulo evidences for association of the survival of motor neuron complex with the fragile X mental retardation protein Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy.Chondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophy A role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophy SMN in spinal muscular atrophy and snRNP biogenesis RNA and disease Dynamic control of Cajal body number during zebrafish embryogenesis.Temporal requirement for SMN in motoneuron development.Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs Inactivation of the SMN complex by oxidative stress Zebrafish as a genetic model in biological and behavioral gerontology: where development meets aging in vertebrates--a mini-review.Spinal Muscular Atrophy: From Defective Chaperoning of snRNP Assembly to Neuromuscular Dysfunction.Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?Proteomic assessment of a cell model of spinal muscular atrophy Specific splicing defects in S. pombe carrying a degron allele of the Survival of Motor Neuron gene SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminals Quality control of assembly-defective U1 snRNAs by decapping and 5'-to-3' exonucleolytic digestion.Transcriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cells GEMINs: potential therapeutic targets for spinal muscular atrophy?A multi-exon-skipping detection assay reveals surprising diversity of splice isoforms of spinal muscular atrophy genes.Depletion of SMN by RNA interference in HeLa cells induces defects in Cajal body formation.Fishing for a mechanism: using zebrafish to understand spinal muscular atrophy.A cell system for phenotypic screening of modifiers of SMN2 gene expression and function.RNA-Seq analysis in mutant zebrafish reveals role of U1C protein in alternative splicing regulation.SMN deficiency alters Nrxn2 expression and splicing in zebrafish and mouse models of spinal muscular atrophy.The Gemin associates of survival motor neuron are required for motor function in Drosophila.ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP.Splicing changes in SMA mouse motoneurons and SMN-depleted neuroblastoma cells: evidence for involvement of splicing regulatory proteins.

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P2860

Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy.

http://www.wikidata.org/entity/Q42548444

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2005 nî lūn-bûn

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Reduced U snRNP assembly cause ...... l for spinal muscular atrophy.

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Reduced U snRNP assembly cause ...... l for spinal muscular atrophy.

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Reduced U snRNP assembly cause ...... l for spinal muscular atrophy.

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Reduced U snRNP assembly cause ...... l for spinal muscular atrophy.

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Genes & Development

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Reduced U snRNP assembly cause ...... l for spinal muscular atrophy.

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Bernhard Laggerbauer

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Christian Eggert

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Christoph Winkler

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Dietmar Gradl

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Doris Wedlich

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Gunter Meister

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Marieke Giegerich

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P2860

A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing

Unique Sm core structure of U7 snRNPs: assembly by a specialized SMN complex and the role of a new component, Lsm11, in histone RNA processing

pICln inhibits snRNP biogenesis by binding core spliceosomal proteins

Symmetrical dimethylation of arginine residues in spliceosomal Sm protein B/B' and the Sm-like protein LSm4, and their interaction with the SMN protein.

The methylosome, a 20S complex containing JBP1 and pICln, produces dimethylarginine-modified Sm proteins

Crystal structures of two Sm protein complexes and their implications for the assembly of the spliceosomal snRNPs

Stages of embryonic development of the zebrafish

Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of spliceosomal Sm proteins

Spliceosomal UsnRNP biogenesis, structure and function

Co-regulation of survival of motor neuron (SMN) protein and its interactor SIP1 during development and in spinal muscular atrophy

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10.1101/GAD.342005

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19

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19

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7561427

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16204184

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spinal muscular atrophy

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1240041

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