Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease.
about
Mood disorders in Huntington's disease: from behavior to cellular and molecular mechanismsRAN translation and frameshifting as translational challenges at simple repeats of human neurodegenerative disordersTransgenic animal models for study of the pathogenesis of Huntington's disease and therapyMouse models of polyglutamine diseases in therapeutic approaches: review and data table. Part II.Huntington's disease: underlying molecular mechanisms and emerging conceptsComparative study of naturally occurring huntingtin fragments in Drosophila points to exon 1 as the most pathogenic species in Huntington's diseaseThe N17 domain mitigates nuclear toxicity in a novel zebrafish Huntington's disease model.Mutant huntingtin gene-dose impacts on aggregate deposition, DARPP32 expression and neuroinflammation in HdhQ150 miceN17 Modifies mutant Huntingtin nuclear pathogenesis and severity of disease in HD BAC transgenic miceHDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegenerationCorrelations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/2 mouse model of HDCorrelations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's diseaseSIRT2 ablation has no effect on tubulin acetylation in brain, cholesterol biosynthesis or the progression of Huntington's disease phenotypes in vivoHDAC4 does not act as a protein deacetylase in the postnatal murine brain in vivoTemporal separation of aggregation and ubiquitination during early inclusion formation in transgenic mice carrying the Huntington's disease mutationGenetic knock-down of HDAC3 does not modify disease-related phenotypes in a mouse model of Huntington's diseaseHdac6 knock-out increases tubulin acetylation but does not modify disease progression in the R6/2 mouse model of Huntington's diseaseA series of N-terminal epitope tagged Hdh knock-in alleles expressing normal and mutant huntingtin: their application to understanding the effect of increasing the length of normal Huntingtin's polyglutamine stretch on CAG140 mouse model pathogenesiSynaptic mutant huntingtin inhibits synapsin-1 phosphorylation and causes neurological symptoms.Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease.Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment.Calpain inhibition mediates autophagy-dependent protection against polyglutamine toxicity.Cholesterol Modifies Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes.The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patientsImpaired adult olfactory bulb neurogenesis in the R6/2 mouse model of Huntington's disease.Ubiquitin-activating enzyme activity contributes to differential accumulation of mutant huntingtin in brain and peripheral tissuesContesting the dogma of an age-related heat shock response impairment: implications for cardiac-specific age-related disorders.Monomeric, oligomeric and polymeric proteins in huntington disease and other diseases of polyglutamine expansionPerturbation with intrabodies reveals that calpain cleavage is required for degradation of huntingtin exon 1Huntingtin localisation studies - a technical review.TR-FRET assays of Huntingtin protein fragments reveal temperature and polyQ length-dependent conformational changes.Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragmentsBifunctional anti-huntingtin proteasome-directed intrabodies mediate efficient degradation of mutant huntingtin exon 1 protein fragments.A coarse-grained model for polyglutamine aggregation modulated by amphipathic flanking sequencesWild-type HTT modulates the enzymatic activity of the neuronal palmitoyl transferase HIP14.Huntington's disease and its therapeutic target genes: a global functional profile based on the HD Research Crossroads databaseUnmasking the roles of N- and C-terminal flanking sequences from exon 1 of huntingtin as modulators of polyglutamine aggregation.Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington's diseaseFragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon agingAnalysis of proteolytic processes and enzymatic activities in the generation of huntingtin n-terminal fragments in an HEK293 cell model.
P2860
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P2860
Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease.
description
2010 nî lūn-bûn
@nan
2010 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Proteolysis of mutant huntingt ...... nuclei in Huntington disease.
@ast
Proteolysis of mutant huntingt ...... nuclei in Huntington disease.
@en
type
label
Proteolysis of mutant huntingt ...... nuclei in Huntington disease.
@ast
Proteolysis of mutant huntingt ...... nuclei in Huntington disease.
@en
prefLabel
Proteolysis of mutant huntingt ...... nuclei in Huntington disease.
@ast
Proteolysis of mutant huntingt ...... nuclei in Huntington disease.
@en
P2093
P2860
P50
P356
P1476
Proteolysis of mutant huntingt ...... nuclei in Huntington disease.
@en
P2093
Alex Osmand
Andreas Weiss
Banghua Sun
Ben Woodman
Christian Landles
Hilary Moffitt
Juliette Gafni
Kirupa Sathasivam
Lisa M Ellerby
Steve Finkbeiner
P2860
P304
P356
10.1074/JBC.M109.075028
P407
P577
2010-01-19T00:00:00Z