Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease.
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Comparison of an expanded ataxia interactome with patient medical records reveals a relationship between macular degeneration and ataxiaPalmitoylation of huntingtin by HIP14 is essential for its trafficking and functionCorticostriatal Dysfunction in Huntington's Disease: The BasicsMouse models of polyglutamine diseases: review and data table. Part IFunctional Differences Between Direct and Indirect Striatal Output Pathways in Huntington's DiseaseA genetic screening strategy identifies novel regulators of the proteostasis networkGenome-wide loss of 5-hmC is a novel epigenetic feature of Huntington's diseaseMice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease.Alterations in striatal synaptic transmission are consistent across genetic mouse models of Huntington's disease.Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation.Characterisation of immune cell function in fragment and full-length Huntington's disease mouse modelsDifferential electrophysiological changes in striatal output neurons in Huntington's disease.Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo.Increased Body Weight of the BAC HD Transgenic Mouse Model of Huntington's Disease Accounts for Some but Not All of the Observed HD-like Motor Deficits.Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease.Differential Changes in Postsynaptic Density Proteins in Postmortem Huntington's Disease and Parkinson's Disease Human Brains.Protein misfolding detected early in pathogenesis of transgenic mouse model of Huntington disease using amyloid seeding assay.Ca(2+) handling in isolated brain mitochondria and cultured neurons derived from the YAC128 mouse model of Huntington's diseaseCharacterization of Behavioral, Neuropathological, Brain Metabolic and Key Molecular Changes in zQ175 Knock-In Mouse Model of Huntington's DiseaseThe group 2 metabotropic glutamate receptor agonist LY379268 rescues neuronal, neurochemical and motor abnormalities in R6/2 Huntington's disease miceSuppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasmExpression of RNAs Coding for Metal Transporters in Blood of Patients with Huntington's DiseaseCompensatory changes in the ubiquitin-proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice partially model Huntington's disease patients.Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice.Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis.Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease.Early defect of transforming growth factor β1 formation in Huntington's disease.Differential ubiquitination and degradation of huntingtin fragments modulated by ubiquitin-protein ligase E3A.Genetic mouse models of Huntington's disease: focus on electrophysiological mechanisms.Molecular mechanisms and potential therapeutical targets in Huntington's disease.Huntington's disease: can mice lead the way to treatment?Screening of therapeutic strategies for Huntington's disease in YAC128 transgenic mice.Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function.PolyQ disease: misfiring of a developmental cell death program?Animal models for metabolic, neuromuscular and ophthalmological rare diseases.The role of the immune system in Huntington's disease.KDM1 histone lysine demethylases as targets for treatments of oncological and neurodegenerative disease.Studying polyglutamine diseases in Drosophila.Cognitive dysfunction in Huntington's disease: mechanisms and therapeutic strategies beyond BDNF.A multifunctional, multi-pathway intracellular localization signal in Huntingtin.
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P2860
Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年学术文章
@wuu
2005年学术文章
@zh
2005年学术文章
@zh-cn
2005年学术文章
@zh-hans
2005年学术文章
@zh-my
2005年学术文章
@zh-sg
2005年學術文章
@yue
2005年學術文章
@zh-hant
name
Selective degeneration and nuc ...... e model of Huntington disease.
@en
Selective degeneration and nuc ...... e model of Huntington disease.
@nl
type
label
Selective degeneration and nuc ...... e model of Huntington disease.
@en
Selective degeneration and nuc ...... e model of Huntington disease.
@nl
prefLabel
Selective degeneration and nuc ...... e model of Huntington disease.
@en
Selective degeneration and nuc ...... e model of Huntington disease.
@nl
P2093
P2860
P356
P1476
Selective degeneration and nuc ...... e model of Huntington disease.
@en
P2093
Elizabeth J Slow
Jeremy M Van Raamsdonk
Zoe Murphy
P2860
P304
P356
10.1093/HMG/DDI407
P577
2005-11-08T00:00:00Z