Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease.
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Huntingtin interacting proteins are genetic modifiers of neurodegenerationTowards a transgenic model of Huntington's disease in a non-human primateRole of cerebral cortex in the neuropathology of Huntington's diseaseMouse models of polyglutamine diseases: review and data table. Part IInhibition of mitochondrial protein import by mutant huntingtinOxygen consumption deficit in Huntington disease mouse brain under metabolic stressMutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitroN-terminal Huntingtin Knock-In Mice: Implications of Removing the N-terminal Region of Huntingtin for Therapy.Knockdown of DISC1 by in utero gene transfer disturbs postnatal dopaminergic maturation in the frontal cortex and leads to adult behavioral deficits.Induction of neostriatal neurogenesis slows disease progression in a transgenic murine model of Huntington diseaseNew insights into mitochondrial structure during cell death.Mouse models of Huntington's disease and methodological considerations for therapeutic trials.N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking.Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms.Tiagabine is neuroprotective in the N171-82Q and R6/2 mouse models of Huntington's diseaseAxon-myelin interactions during a viral infection of the central nervous systemImpaired adult olfactory bulb neurogenesis in the R6/2 mouse model of Huntington's disease.Inhibitors of protein disulfide isomerase suppress apoptosis induced by misfolded proteins.Huntington disease models and human neuropathology: similarities and differencesMutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease miceEfficacy of fumaric acid esters in the R6/2 and YAC128 models of Huntington's disease.Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions.Early autophagic response in a novel knock-in model of Huntington diseaseExpression of Huntington's disease protein results in apoptotic neurons in the brains of cloned transgenic pigsHuntingtin fragments and SOD1 mutants form soluble oligomers in the cellLack of interleukin-1 type 1 receptor enhances the accumulation of mutant huntingtin in the striatum and exacerbates the neurological phenotypes of Huntington's disease mice.Neuroprotective properties of cannabigerol in Huntington's disease: studies in R6/2 mice and 3-nitropropionate-lesioned mice.Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation.Viral delivery of glial cell line-derived neurotrophic factor improves behavior and protects striatal neurons in a mouse model of Huntington's diseaseCleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo.Striatal infusion of glial conditioned medium diminishes huntingtin pathology in r6/1 mice.Biophysical significance of the inner mitochondrial membrane structure on the electrochemical potential of mitochondria.Aged monkey brains reveal the role of ubiquitin-conjugating enzyme UBE2N in the synaptosomal accumulation of mutant huntingtin.Conditional BDNF release under pathological conditions improves Huntington's disease pathology by delaying neuronal dysfunction.Altered excitatory and inhibitory inputs to striatal medium-sized spiny neurons and cortical pyramidal neurons in the Q175 mouse model of Huntington's disease.CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse.Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's diseaseTranslating therapies for Huntington's disease from genetic animal models to clinical trials.Novel BAC Mouse Model of Huntington's Disease with 225 CAG Repeats Exhibits an Early Widespread and Stable Degenerative Phenotype.Interrogation of brain miRNA and mRNA expression profiles reveals a molecular regulatory network that is perturbed by mutant huntingtin.
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Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease.
description
2003 nî lūn-bûn
@nan
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
2003年论文
@zh
2003年论文
@zh-cn
name
Mutant huntingtin causes conte ...... ice with Huntington's disease.
@en
type
label
Mutant huntingtin causes conte ...... ice with Huntington's disease.
@en
prefLabel
Mutant huntingtin causes conte ...... ice with Huntington's disease.
@en
P2093
P1476
Mutant huntingtin causes conte ...... mice with Huntington's disease
@en
P2093
Ajay Pillarisetti
Shi-Hua Li
Xiao-Jiang Li
Zhao-Xue Yu
P304
P356
10.1523/JNEUROSCI.23-06-02193.2003
P407
P577
2003-03-01T00:00:00Z