Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
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Ubiquitin-proteasome system involvement in Huntington's diseaseThe Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcriptionRhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicityInteraction of Huntington disease protein with transcriptional activator Sp1Cdc42-interacting protein 4 binds to huntingtin: neuropathologic and biological evidence for a role in Huntington's diseaseThe Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesisHuman single-chain Fv intrabodies counteract in situ huntingtin aggregation in cellular models of Huntington's diseaseTowards a transgenic model of Huntington's disease in a non-human primateIntracellular degradation of misfolded proteins in polyglutamine neurodegenerative diseasesAsialoerythropoietin is not effective in the R6/2 line of Huntington's disease miceSubcellular Clearance and Accumulation of Huntington Disease Protein: A Mini-ReviewDopamine Receptors and NeurodegenerationTransgenic animal models for study of the pathogenesis of Huntington's disease and therapyModeling Huntington's disease with induced pluripotent stem cellsGenetics and neuropathology of Huntington's diseaseComparative 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-myogenin axis as an important marker of HD-related skeletal muscle atrophyHDAC4 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 HDA genetic screening strategy identifies novel regulators of the proteostasis networkIdentification of tissue transglutaminase-reactive lysine residues in glyceraldehyde-3-phosphate dehydrogenaseSmall changes huge impact: the role of protein posttranslational modifications in cellular homeostasis and diseaseUnraveling Comparative Anti-Amyloidogenic Behavior of Pyrazinamide and D-Cycloserine: A Mechanistic Biophysical InsightMutant huntingtin alters cell fate in response to microtubule depolymerization via the GEF-H1-RhoA-ERK pathwayTemporal separation of aggregation and ubiquitination during early inclusion formation in transgenic mice carrying the Huntington's disease mutationInhibition of the striatal specific phosphodiesterase PDE10A ameliorates striatal and cortical pathology in R6/2 mouse model of Huntington's diseaseSynchrotron infrared microspectroscopy detecting the evolution of Huntington's disease neuropathology and suggesting unique correlates of dysfunction in white versus gray brain matter.A 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 pathogenesiIKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.Synaptic mutant huntingtin inhibits synapsin-1 phosphorylation and causes neurological symptoms.Mouse models of Huntington's disease and methodological considerations for therapeutic trials.N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking.Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo.SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776.Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment.Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative.Ubiquitination is involved in secondary growth, not initial formation of polyglutamine protein aggregates in C. elegans.
P2860
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P2860
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
description
1999 nî lūn-bûn
@nan
1999年の論文
@ja
1999年学术文章
@wuu
1999年学术文章
@zh
1999年学术文章
@zh-cn
1999年学术文章
@zh-hans
1999年学术文章
@zh-my
1999年学术文章
@zh-sg
1999年學術文章
@yue
1999年學術文章
@zh-hant
name
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
@en
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
@nl
type
label
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
@en
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
@nl
prefLabel
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
@en
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
@nl
P2093
P1476
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology
@en
P2093
C A Gutekunst
J S Mulroy
R J Ferrante
S Kuemmerle
S M Hersch
P304
P356
10.1523/JNEUROSCI.19-07-02522.1999
P407
P577
1999-04-01T00:00:00Z