| Q35022497 | A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila |
| Q38974551 | A link between chromatin condensation mechanisms and Huntington's disease: connecting the dots. |
| Q64882921 | A patient-derived cellular model for Huntington's disease reveals phenotypes at clinically relevant CAG lengths. |
| Q34657321 | A phenotypic screening assay for modulators of huntingtin-induced transcriptional dysregulation |
| Q30578694 | A potent and selective Sirtuin 1 inhibitor alleviates pathology in multiple animal and cell models of Huntington's disease |
| Q38038981 | An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity |
| Q52571894 | Analysis of oxidative events induced by expanded polyglutamine huntingtin exon 1 that are differentially restored by expression of heat shock proteins or treatment with an antioxidant. |
| Q36606399 | Animal models of Huntington's disease: implications in uncovering pathogenic mechanisms and developing therapies |
| Q42473221 | Antioxidants can inhibit basal autophagy and enhance neurodegeneration in models of polyglutamine disease. |
| Q35097613 | Are Huntington's and polyglutamine-based ataxias proteasome storage diseases? |
| Q43755141 | Autophagic clearance of aggregate-prone proteins associated with neurodegeneration. |
| Q43284919 | BAG1 modulates huntingtin toxicity, aggregation, degradation, and subcellular distribution |
| Q45292425 | CRE-mediated transcription is increased in Huntington's disease transgenic mice. |
| Q45307343 | Cells exposed to a huntingtin fragment containing an expanded polyglutamine tract show no sign of ion channel formation: results arguing against the ion channel hypothesis |
| Q64074925 | Chemical Screening Approaches Enabling Drug Discovery of Autophagy Modulators for Biomedical Applications in Human Diseases |
| Q33258207 | Cholinergic neuronal defect without cell loss in Huntington's disease |
| Q84346351 | Clearance of mutant huntingtin |
| Q45295157 | DJ-1 modulates aggregation and pathogenesis in models of Huntington's disease. |
| Q44662096 | Decreased cAMP response element-mediated transcription: an early event in exon 1 and full-length cell models of Huntington's disease that contributes to polyglutamine pathogenesis |
| Q28479035 | Decreased striatal RGS2 expression is neuroprotective in Huntington's disease (HD) and exemplifies a compensatory aspect of HD-induced gene regulation |
| Q42790645 | Dendritic spine loss and neurodegeneration is rescued by Rab11 in models of Huntington's disease |
| Q45304103 | Differential morphology and composition of inclusions in the R6/2 mouse and PC12 cell models of Huntington's disease. |
| Q35294913 | Direct reprogramming of Huntington's disease patient fibroblasts into neuron-like cells leads to abnormal neurite outgrowth, increased cell death, and aggregate formation |
| Q40526299 | Disrupted spermine homeostasis: a novel mechanism in polyglutamine-mediated aggregation and cell death. |
| Q34124865 | Disruption of CREB function in brain leads to neurodegeneration |
| Q34618914 | Does Huntingtin play a role in selective macroautophagy? |
| Q28203597 | Dysfunction of wild-type huntingtin in Huntington disease |
| Q41822886 | Early and late events induced by polyQ-expanded proteins: identification of a common pathogenic property of polYQ-expanded proteins |
| Q39335116 | Early transcriptional changes linked to naturally occurring Huntington's disease mutations in neural derivatives of human embryonic stem cells. |
| Q39010713 | Expansion of the polyQ repeats in THAP11 forms intranuclear aggregation and causes cell G0/G1 arrest |
| Q36742104 | Expression of expanded polyglutamine targets profilin for degradation and alters actin dynamics |
| Q28179222 | Expression of mutant huntingtin blocks exocytosis in PC12 cells by depletion of complexin II |
| Q29619559 | Function and regulation of CREB family transcription factors in the nervous system |
| Q41557401 | Functional gene expression profiling in yeast implicates translational dysfunction in mutant huntingtin toxicity. |
| Q39772092 | Fused 3-Hydroxy-3-trifluoromethylpyrazoles Inhibit Mutant Huntingtin Toxicity |
| Q33811254 | Geldanamycin attenuates 3‑nitropropionic acid‑induced apoptosis and JNK activation through the expression of HSP 70 in striatal cells |
| Q40623717 | Gene Expression Profiling in Ataxin-3 Expressing Cell Lines Reveals Distinct Effects of Normal and Mutant Ataxin-3 |
| Q36954090 | Generic cell dysfunction in neurodegenerative disorders: role of surfaces in early protein misfolding, aggregation, and aggregate cytotoxicity. |
| Q35764318 | Genetic and pharmacological suppression of polyglutamine-dependent neuronal dysfunction in Caenorhabditis elegans |
| Q34029504 | Genome wide gene expression regulation by HIP1 Protein Interactor, HIPPI: prediction and validation |
| Q52651349 | Glial and neuronal expression of polyglutamine proteins induce behavioral changes and aggregate formation in Drosophila. |
| Q45306368 | Glycogen synthase kinase-3beta inhibitors prevent cellular polyglutamine toxicity caused by the Huntington's disease mutation |
| Q36829093 | Heat shock proteins in the retina: Focus on HSP70 and alpha crystallins in ganglion cell survival |
| Q39229625 | Heat shock transcription factor-1 suppresses apoptotic cell death and ROS generation in 3-nitropropionic acid-stimulated striatal cells |
| Q34274478 | Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis. |
| Q35158643 | Huntington's Disease: From Mutant Huntingtin Protein to Neurotrophic Factor Therapy |
| Q33193898 | Huntington's disease: a synaptopathy? |
| Q34747269 | Identification of an allosteric small-molecule inhibitor selective for the inducible form of heat shock protein 70. |
| Q36159463 | Impaired GAPDH-induced mitophagy contributes to the pathology of Huntington's disease |
| Q39885837 | Inducible mutant huntingtin expression in HN10 cells reproduces Huntington's disease-like neuronal dysfunction |
| Q33878287 | Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments |
| Q28261987 | Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease |
| Q28748849 | Inhibition of the striatal specific phosphodiesterase PDE10A ameliorates striatal and cortical pathology in R6/2 mouse model of Huntington's disease |
| Q46442462 | Interference of CREB-dependent transcriptional activation by expanded polyglutamine stretches--augmentation of transcriptional activation as a potential therapeutic strategy for polyglutamine diseases |
| Q41807512 | Is modulating translation a therapeutic option for Huntington's disease? |
| Q35777504 | Kennedy's disease: pathogenesis and clinical approaches |
| Q34586943 | Lessons from animal models of Huntington's disease |
| Q36321015 | Lithium induces autophagy by inhibiting inositol monophosphatase |
| Q46430923 | Loss of PINK1 function affects development and results in neurodegeneration in zebrafish |
| Q38085375 | Lysine acetyltransferases CBP and p300 as therapeutic targets in cognitive and neurodegenerative disorders. |
| Q35105770 | MicroRNAs located in the Hox gene clusters are implicated in huntington's disease pathogenesis. |
| Q36348804 | Microtubule destabilization and nuclear entry are sequential steps leading to toxicity in Huntington's disease |
| Q28580488 | Mitochondrial cyclic AMP response element-binding protein (CREB) mediates mitochondrial gene expression and neuronal survival |
| Q33580327 | Mitochondrial matters of the brain: the role in Huntington's disease |
| Q94186808 | Modulation of Huntingtin Toxicity by BAG1 is Dependent on an Intact BAG Domain |
| Q33526350 | Modulation of heat shock transcription factor 1 as a therapeutic target for small molecule intervention in neurodegenerative disease |
| Q34443867 | Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity |
| Q41600591 | Mutant huntingtin activates Nrf2-responsive genes and impairs dopamine synthesis in a PC12 model of Huntington's disease |
| Q64273045 | Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription |
| Q28584697 | Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro |
| Q44473475 | Mutant huntingtin increases nuclear corepressor function and enhances ligand-dependent nuclear hormone receptor activation |
| Q92269192 | Neuroprotective Effects of Doxycycline in the R6/2 Mouse Model of Huntington's Disease |
| Q44163255 | No correlation between aggregates of Cu/Zn superoxide dismutase and cell death in familial amyotrophic lateral sclerosis |
| Q37189443 | Non-targeted identification of prions and amyloid-forming proteins from yeast and mammalian cells |
| Q28593520 | Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity |
| Q44931209 | Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice |
| Q43207028 | PRMT5- mediated symmetric arginine dimethylation is attenuated by mutant huntingtin and is impaired in Huntington's disease (HD). |
| Q35550999 | Perturbed signal transduction in neurodegenerative disorders involving aberrant protein aggregation |
| Q34114458 | Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease |
| Q63951832 | Plans for HDBase—a research community website for Huntington's Disease |
| Q24656119 | Polyalanine and polyserine frameshift products in Huntington's disease |
| Q34225700 | Protein aggregation and aggregate toxicity: new insights into protein folding, misfolding diseases and biological evolution |
| Q37007345 | Protein folding and aggregation into amyloid: the interference by natural phenolic compounds |
| Q28571475 | Protein kinase A regulates molecular chaperone transcription and protein aggregation |
| Q44133868 | Protein tyrosine phosphatases are up-regulated and participate in cell death induced by polyglutamine expansion |
| Q35758594 | Proteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates. |
| Q34303837 | RNAi-based therapies for Huntington's disease: delivery challenges and opportunities |
| Q37083085 | Rab5 modulates aggregation and toxicity of mutant huntingtin through macroautophagy in cell and fly models of Huntington disease |
| Q45301357 | Regulation of intracellular trafficking of huntingtin-associated protein-1 is critical for TrkA protein levels and neurite outgrowth. |
| Q47732711 | Role of Phosphodiesterases in Huntington's Disease |
| Q35764321 | Role of heat shock proteins during polyglutamine neurodegeneration: mechanisms and hypothesis |
| Q24310080 | Rrs1 is involved in endoplasmic reticulum stress response in Huntington disease |
| Q38616832 | Selective Sparing of Striatal Interneurons after Poly (ADP-Ribose) Polymerase 1 Inhibition in the R6/2 Mouse Model of Huntington's Disease |
| Q36597920 | Selective neuronal degeneration in Huntington's disease |
| Q35683088 | Small-molecule proteostasis regulators for protein conformational diseases |
| Q47232763 | Spatiotemporal Proteomic Profiling of Huntington's Disease Inclusions Reveals Widespread Loss of Protein Function. |
| Q64106672 | Squaramide-based synthetic chloride transporters activate TFEB but block autophagic flux |
| Q36320449 | Structural properties and neuronal toxicity of amyotrophic lateral sclerosis-associated Cu/Zn superoxide dismutase 1 aggregates. |
| Q40175911 | Systematic uncovering of multiple pathways underlying the pathology of Huntington disease by an acid-cleavable isotope-coded affinity tag approach |
| Q45298693 | TAK-063, a Novel Phosphodiesterase 10A Inhibitor, Protects from Striatal Neurodegeneration and Ameliorates Behavioral Deficits in the R6/2 Mouse Model of Huntington's Disease |
| Q35095142 | Targeting aggregation in the development of therapeutics for the treatment of Huntington's disease and other polyglutamine repeat diseases |
| Q28728849 | Temporal separation of aggregation and ubiquitination during early inclusion formation in transgenic mice carrying the Huntington's disease mutation |
| Q37408287 | The Association of VDAC with Cell Viability of PC12 Model of Huntington's Disease. |
| Q88683651 | The disorderly conduct of Hsc70 and its interaction with the Alzheimer's-related Tau protein |
| Q36370547 | The dynamics of early-state transcriptional changes and aggregate formation in a Huntington's disease cell model |
| Q34308028 | The energetics of Huntington's disease |
| Q28580600 | The induction levels of heat shock protein 70 differentiate the vulnerabilities to mutant huntingtin among neuronal subtypes |
| Q35024695 | Transcription, epigenetics and ameliorative strategies in Huntington's Disease: a genome-wide perspective |
| Q35113363 | Transcriptional abnormalities in Huntington disease |
| Q55497345 | Transcriptional profiling and biomarker identification reveal tissue specific effects of expanded ataxin-3 in a spinocerebellar ataxia type 3 mouse model. |
| Q36752799 | Transcriptional signatures in Huntington's disease |
| Q39631653 | Transcriptome sequencing reveals aberrant alternative splicing in Huntington's disease |
| Q38318433 | Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophy |
| Q45303665 | Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein. |
| Q35025253 | Trinucleotide repeat disease. The androgen receptor in spinal and bulbar muscular atrophy |
| Q34658555 | Wild-type but not mutant huntingtin modulates the transcriptional activity of liver X receptors |
| Q45303717 | Wild-type huntingtin participates in protein trafficking between the Golgi and the extracellular space |
| Q33900572 | cAMP-response element-binding protein and heat-shock protein 70 additively suppress polyglutamine-mediated toxicity in Drosophila |
| Q45250028 | cAMP-response element-binding protein contributes to suppression of the A2A adenosine receptor promoter by mutant Huntingtin with expanded polyglutamine residues |
| Q37535111 | miRNAs: Key Players in Neurodegenerative Disorders and Epilepsy |