siRNA knock-down of mutant torsinA restores processing through secretory pathway in DYT1 dystonia cells.
about
The early-onset torsion dystonia-associated protein, torsinA, displays molecular chaperone activity in vitroTorsinA binds the KASH domain of nesprins and participates in linkage between nuclear envelope and cytoskeletonTorsins: not your typical AAA+ ATPasesTorsinA hypofunction causes abnormal twisting movements and sensorimotor circuit neurodegenerationChemical enhancement of torsinA function in cell and animal models of torsion dystonia.Mutant torsinA interacts with tyrosine hydroxylase in cultured cells.Convergent evidence for abnormal striatal synaptic plasticity in dystonia.Biochemical and cellular analysis of human variants of the DYT1 dystonia protein, TorsinA/TOR1AThe early-onset torsion dystonia-associated protein, torsinA, is a homeostatic regulator of endoplasmic reticulum stress response.Current Gaps in the Understanding of the Subcellular Distribution of Exogenous and Endogenous Protein TorsinA.4-Phenylbutyrate attenuates the ER stress response and cyclic AMP accumulation in DYT1 dystonia cell models.Untethering the nuclear envelope and cytoskeleton: biologically distinct dystonias arising from a common cellular dysfunctionTorsin A Localization in the Mouse Cerebellar Synaptic Circuitry.Static retention of the lumenal monotopic membrane protein torsinA in the endoplasmic reticulum.Inherited isolated dystonia: clinical genetics and gene function.The pathogenic human Torsin A in Drosophila activates the unfolded protein response and increases susceptibility to oxidative stress.Forebrain deletion of the dystonia protein torsinA causes dystonic-like movements and loss of striatal cholinergic neurons.The ubiquitin ligase F-box/G-domain protein 1 promotes the degradation of the disease-linked protein torsinA through the ubiquitin-proteasome pathway and macroautophagy.TorsinA participates in endoplasmic reticulum-associated degradationIdentifying the genetic components underlying the pathophysiology of movement disorders.Gaussia luciferase reporter assay for monitoring biological processes in culture and in vivoDisruption of Protein Processing in the Endoplasmic Reticulum of DYT1 Knock-in Mice Implicates Novel Pathways in Dystonia Pathogenesis.Interaction of torsinA with its major binding partners is impaired by the dystonia-associated DeltaGAG deletion.Primary dystonia: molecules and mechanisms.Lentiviral vector-mediated gene transfer and RNA silencing technology in neuronal dysfunctions.Molecular pathways in dystonia.Emerging common molecular pathways for primary dystonia.Early-onset torsion dystonia: a novel high-throughput yeast genetic screen for factors modifying protein levels of torsinAΔE.A functional role for TorsinA in herpes simplex virus 1 nuclear egress.Membrane defects and genetic redundancy: Are we at a turning point for DYT1 dystonia?Transcriptional and proteomic profiling in a cellular model of DYT1 dystoniaLethal toxicity caused by expression of shRNA in the mouse striatum: implications for therapeutic design.Allele-specific RNA interference in FOP silencing the FOP gene.Functional Genomic Analyses of Mendelian and Sporadic Disease Identify Impaired eIF2α Signaling as a Generalizable Mechanism for Dystonia.TorsinA dysfunction causes persistent neuronal nuclear pore defects.
P2860
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P2860
siRNA knock-down of mutant torsinA restores processing through secretory pathway in DYT1 dystonia cells.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
siRNA knock-down of mutant tor ...... athway in DYT1 dystonia cells.
@en
type
label
siRNA knock-down of mutant tor ...... athway in DYT1 dystonia cells.
@en
prefLabel
siRNA knock-down of mutant tor ...... athway in DYT1 dystonia cells.
@en
P2093
P2860
P356
P1476
siRNA knock-down of mutant tor ...... athway in DYT1 dystonia cells.
@en
P2093
Bakhos A Tannous
Brian Niland
Dinah W Y Sah
Flávia C Nery
Jeffrey W Hewett
Pamela Tan
Philipp Hadwiger
Xandra O Breakefield
P2860
P304
P356
10.1093/HMG/DDN032
P577
2008-02-07T00:00:00Z