| review article | Q7318358 |
| scholarly article | Q13442814 |
| P2093 | author name string | Steven Finkbeiner | |
| P2860 | cites work | Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract | Q73561135 |
| Oligomerization of expanded-polyglutamine domain fluorescent fusion proteins in cultured mammalian cells | Q73703151 | ||
| Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial? | Q77430975 | ||
| Polyglutamine protein aggregates are dynamic | Q78315034 | ||
| A toxic monomeric conformer of the polyglutamine protein | Q79976767 | ||
| Functional amyloid formation within mammalian tissue | Q21092782 | ||
| CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation | Q24296740 | ||
| The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress | Q24301895 | ||
| Sequestosome 1/p62 shuttles polyubiquitinated tau for proteasomal degradation | Q24305254 | ||
| Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity | Q24317318 | ||
| HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin | Q24337155 | ||
| Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis. | Q24534867 | ||
| Aging as an event of proteostasis collapse | Q24630961 | ||
| Secondary Structure of Huntingtin Amino-Terminal Region | Q27657408 | ||
| A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. | Q27860836 | ||
| Hsp104, Hsp70, and Hsp40: a novel chaperone system that rescues previously aggregated proteins | Q27931364 | ||
| Protein disaggregation mediated by heat-shock protein Hsp104. | Q27940314 | ||
| Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo | Q28131779 | ||
| Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity | Q28143597 | ||
| The ubiquitin-related BAG-1 provides a link between the molecular chaperones Hsc70/Hsp70 and the proteasome | Q28144546 | ||
| Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation | Q28246858 | ||
| Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1 | Q28272543 | ||
| RNA toxicity is a component of ataxin-3 degeneration in Drosophila | Q28278448 | ||
| Pathway for degradation of peptides generated by proteasomes: a key role for thimet oligopeptidase and other metallopeptidases | Q28278713 | ||
| Neurodegenerative disease: neuron protection agency | Q28287753 | ||
| Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death | Q28287762 | ||
| Aggresomes: a cellular response to misfolded proteins | Q28292275 | ||
| HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS | Q28306195 | ||
| Huntington's disease: seeing the pathogenic process through a genetic lens | Q36531069 | ||
| Disease-modifying pathways in neurodegeneration. | Q36621175 | ||
| Detection of Huntington's disease decades before diagnosis: the Predict-HD study | Q36941187 | ||
| The autophagy-lysosomal degradation pathway: role in neurodegenerative disease and therapy. | Q36991909 | ||
| The ubiquitin-proteasome pathway and proteasome inhibitors | Q28367002 | ||
| Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease | Q28588314 | ||
| Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis | Q29547501 | ||
| Impairment of the ubiquitin-proteasome system by protein aggregation | Q29614556 | ||
| Twenty years of the Alzheimer's disease amyloid hypothesis: a genetic perspective | Q29614702 | ||
| Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice | Q29615357 | ||
| Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain | Q29617982 | ||
| Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy | Q29622819 | ||
| Aberrant excitatory neuronal activity and compensatory remodeling of inhibitory hippocampal circuits in mouse models of Alzheimer's disease | Q30080016 | ||
| Extended polyglutamine tracts cause aggregation and structural perturbation of an adjacent beta barrel protein | Q30159881 | ||
| Native Functions of the Androgen Receptor Are Essential to Pathogenesis in a Drosophila Model of Spinobulbar Muscular Atrophy | Q30423500 | ||
| Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models | Q30483013 | ||
| SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776. | Q30496777 | ||
| Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells | Q30779186 | ||
| Automated microscope system for determining factors that predict neuronal fate. | Q30856679 | ||
| Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis | Q30986761 | ||
| Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration | Q30992818 | ||
| Prevention of polyglutamine oligomerization and neurodegeneration by the peptide inhibitor QBP1 in Drosophila | Q31142912 | ||
| Gametic but not somatic instability of CAG repeat length in Huntington's disease. | Q33596051 | ||
| Intracellular inclusions, pathological markers in diseases caused by expanded polyglutamine tracts? | Q33607537 | ||
| The chaperonin TRiC controls polyglutamine aggregation and toxicity through subunit-specific interactions | Q33693921 | ||
| Genome-wide RNA interference screen identifies previously undescribed regulators of polyglutamine aggregation | Q33694463 | ||
| Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice. | Q33713689 | ||
| Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone | Q33858200 | ||
| Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment. | Q33894384 | ||
| Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions | Q33914031 | ||
| Autophagy, cytoplasm-to-vacuole targeting pathway, and pexophagy in yeast and mammalian cells. | Q33916012 | ||
| Unraveling a role for dopamine in Huntington's disease: the dual role of reactive oxygen species and D2 receptor stimulation | Q33922831 | ||
| RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model | Q33936969 | ||
| Juvenile onset Huntington's disease--clinical and research perspectives. | Q34091006 | ||
| Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation | Q34154622 | ||
| Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course | Q34181024 | ||
| Caretaker or undertaker? The role of the proteasome in aging | Q34224564 | ||
| Yeast genes that enhance the toxicity of a mutant huntingtin fragment or alpha-synuclein | Q34281429 | ||
| Proteasomes and proteasome inhibition in the central nervous system | Q34346616 | ||
| Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice | Q37072467 | ||
| Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset | Q37073913 | ||
| Single neuron ubiquitin-proteasome dynamics accompanying inclusion body formation in huntington disease | Q37094175 | ||
| Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins | Q37109961 | ||
| Autophagy-mediated clearance of aggresomes is not a universal phenomenon | Q37294767 | ||
| Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease | Q37336253 | ||
| Huntingtin aggregation monitored by dynamic light scattering | Q37381423 | ||
| Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease | Q37471751 | ||
| Inefficient degradation of truncated polyglutamine proteins by the proteasome | Q37592772 | ||
| Dopamine receptor gene expression by enkephalin neurons in rat forebrain. | Q37662769 | ||
| Preferential loss of preproenkephalin versus preprotachykinin neurons from the striatum of Huntington's disease patients | Q38288534 | ||
| Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation | Q38720406 | ||
| PolyQ Disease: Too Many Qs, Too Much Function? | Q38791237 | ||
| Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic | Q40065738 | ||
| Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation. | Q40461698 | ||
| A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure. | Q40462323 | ||
| Aging, energy, and oxidative stress in neurodegenerative diseases. | Q40478731 | ||
| Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions. | Q40513609 | ||
| Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation | Q40540661 | ||
| Hsp70 and aging | Q40648794 | ||
| Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein | Q40662036 | ||
| Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival. | Q40758147 | ||
| CREB-binding protein sequestration by expanded polyglutamine | Q40859094 | ||
| Evidence for a recruitment and sequestration mechanism in Huntington's disease | Q40859700 | ||
| Aggregation of truncated GST-HD exon 1 fusion proteins containing normal range and expanded glutamine repeats | Q40859885 | ||
| Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro | Q40968069 | ||
| Protein conformation and disease. | Q41122642 | ||
| Absence of disease phenotype and intergenerational stability of the CAG repeat in transgenic mice expressing the human Huntington disease transcript | Q41231403 | ||
| Molecular chaperones: clasping the prize | Q41311924 | ||
| Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases? | Q41680087 | ||
| Intranuclear inclusions and the ubiquitin-proteasome pathway: digestion of a red herring? | Q41712647 | ||
| Genetic suppression of polyglutamine toxicity in Drosophila | Q41724127 | ||
| p62 Is a common component of cytoplasmic inclusions in protein aggregation diseases | Q42184099 | ||
| Immunocytochemical studies of substance P and leucine-enkephalin in Huntington's disease | Q42449413 | ||
| Somatic and gonadal mosaicism of the Huntington disease gene CAG repeat in brain and sperm. | Q42496337 | ||
| Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage | Q42512972 | ||
| Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein | Q42598739 | ||
| Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice | Q43670927 | ||
| Solution structure of polyglutamine tracts in GST-polyglutamine fusion proteins | Q43923832 | ||
| Expanded polyglutamine stretches form an 'aggresome'. | Q43963278 | ||
| Amyloid-like features of polyglutamine aggregates and their assembly kinetics | Q44014104 | ||
| The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt. | Q44025697 | ||
| Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells | Q44189907 | ||
| Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. | Q44286601 | ||
| SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity. | Q44307899 | ||
| Emerging role for autophagy in the removal of aggresomes in Schwann cells. | Q44662067 | ||
| Inhibition of polyglutamine aggregate cytotoxicity by a structure-based elongation inhibitor | Q44786911 | ||
| Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins | Q44833524 | ||
| Proteasome degrades soluble expanded polyglutamine completely and efficiently | Q44944511 | ||
| Microtubule disruption inhibits autophagosome-lysosome fusion: implications for studying the roles of aggresomes in polyglutamine diseases | Q45027531 | ||
| Permeabilization of lipid bilayers is a common conformation-dependent activity of soluble amyloid oligomers in protein misfolding diseases | Q45072759 | ||
| Somatic mosaicism in sperm is associated with intergenerational (CAG)n changes in Huntington disease. | Q45288509 | ||
| Molecular analysis of juvenile Huntington disease: the major influence on (CAG)n repeat length is the sex of the affected parent. | Q45290342 | ||
| Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease | Q45290871 | ||
| Autophagy regulates the processing of amino terminal huntingtin fragments | Q45291371 | ||
| Trinucleotide repeat length and clinical progression in Huntington's disease | Q45291777 | ||
| Reduction in enkephalin and substance P messenger RNA in the striatum of early grade Huntington's disease: a detailed cellular in situ hybridization study | Q45292432 | ||
| A new hypothesis of neurodegenerative diseases: the deleterious network hypothesis | Q45293309 | ||
| Evidence of cortical metabolic dysfunction in early Huntington's disease by single-photon-emission computed tomography | Q45293340 | ||
| Differential expression of normal and mutant Huntington's disease gene alleles | Q45293594 | ||
| Neurodegeneration: aging and dementia. Etiopathogenic role of electron transport disorders. Therapeutic possibilities | Q45293983 | ||
| PET study of the pre- and post-synaptic dopaminergic markers for the neurodegenerative process in Huntington's disease | Q45294163 | ||
| CAG repeat number governs the development rate of pathology in Huntington's disease | Q45294278 | ||
| Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo | Q45294913 | ||
| Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? | Q45295535 | ||
| Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation | Q45295822 | ||
| Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length | Q45296163 | ||
| Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. | Q45296540 | ||
| Acute polyglutamine expression in inducible mouse model unravels ubiquitin/proteasome system impairment and permanent recovery attributable to aggregate formation. | Q45296946 | ||
| Neuropathological classification of Huntington's disease | Q45297167 | ||
| Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. | Q45297497 | ||
| History of genetic disease: the molecular genetics of Huntington disease - a history | Q45298333 | ||
| Regional cortical thinning in preclinical Huntington disease and its relationship to cognition | Q45298420 | ||
| Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins | Q45298596 | ||
| Overexpression of yeast hsp104 reduces polyglutamine aggregation and prolongs survival of a transgenic mouse model of Huntington's disease | Q45298652 | ||
| CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. | Q45298680 | ||
| Clinical and neuropathologic assessment of severity in Huntington's disease | Q45298780 | ||
| Proteasome impairment does not contribute to pathogenesis in R6/2 Huntington's disease mice: exclusion of proteasome activator REGgamma as a therapeutic target | Q45299199 | ||
| Huntington aggregates may not predict neuronal death in Huntington's disease | Q45299493 | ||
| A one-hit model of cell death in inherited neuronal degenerations | Q45300872 | ||
| Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy. | Q45301281 | ||
| Inhibition of 26S proteasome activity by huntingtin filaments but not inclusion bodies isolated from mouse and human brain | Q45301604 | ||
| Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein. | Q45303665 | ||
| Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice | Q45305847 | ||
| Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis | Q45307235 | ||
| Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer | Q46229904 | ||
| Sodium dodecyl sulfate-insoluble oligomers are involved in polyglutamine degeneration. | Q46535359 | ||
| ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology | Q47072817 | ||
| Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes | Q48081281 | ||
| Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. | Q48373564 | ||
| Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice | Q48373570 | ||
| ApoE isoform-specific effects on LTP: blockade by oligomeric amyloid-beta1-42. | Q49149877 | ||
| Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers. | Q50715387 | ||
| Involvement of macroautophagy in the dissolution of neuronal inclusions. | Q50788486 | ||
| Characterizing the conformational ensemble of monomeric polyglutamine. | Q51317035 | ||
| Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state. | Q52573641 | ||
| Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. | Q52574703 | ||
| Membrane filter assay for detection of amyloid-like polyglutamine-containing protein aggregates. | Q52976682 | ||
| The ubiquitin-proteasome pathway in Huntington's disease. | Q55433686 | ||
| Pathogenic and Non-pathogenic Polyglutamine Tracts Have Similar Structural Properties: Towards a Length-dependent Toxicity Gradient | Q57387347 | ||
| Trinucleotide repeat length instability and age of onset in Huntington's disease | Q34357407 | ||
| Progressive disruption of cellular protein folding in models of polyglutamine diseases. | Q34493281 | ||
| Autophagy and aging: the importance of maintaining "clean" cells | Q34552013 | ||
| Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases | Q34573066 | ||
| Small molecules enhance autophagy and reduce toxicity in Huntington's disease models. | Q34626231 | ||
| Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron | Q34797015 | ||
| Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin | Q35013054 | ||
| Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease | Q35084195 | ||
| Are Huntington's and polyglutamine-based ataxias proteasome storage diseases? | Q35097613 | ||
| Presymptomatic compensation in Parkinson's disease is not dopamine-mediated | Q35104797 | ||
| Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology | Q35134776 | ||
| Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils | Q35169533 | ||
| Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates | Q35171105 | ||
| Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease | Q35212735 | ||
| The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg. | Q35558459 | ||
| Recent advances in understanding the pathogenesis of polyglutamine diseases: involvement of molecular chaperones and ubiquitin-proteasome pathway. | Q35576413 | ||
| Anticipation and instability of IT-15 (CAG)n repeats in parent-offspring pairs with Huntington disease | Q35643575 | ||
| Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans | Q35746858 | ||
| Network-level neuroplasticity in cortico-basal ganglia pathways | Q35804732 | ||
| Mechanism of neurodegenerative disease: role of the ubiquitin proteasome system | Q35821609 | ||
| Autophagy, proteasomes, lipofuscin, and oxidative stress in the aging brain. | Q35869366 | ||
| Regulation and role of autophagy in mammalian cells | Q35869384 | ||
| Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats | Q35882893 | ||
| A modified beta-amyloid hypothesis: intraneuronal accumulation of the beta-amyloid peptide--the first step of a fatal cascade | Q35918740 | ||
| Can autophagy protect against neurodegeneration caused by aggregate-prone proteins? | Q35945776 | ||
| Modulation of neurodegeneration by molecular chaperones | Q35990274 | ||
| The aggravating role of the ubiquitin-proteasome system in neurodegeneration | Q36020204 | ||
| Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits | Q36089160 | ||
| Autophagy-mediated clearance of huntingtin aggregates triggered by the insulin-signaling pathway | Q36117201 | ||
| Pathological synaptic plasticity in the striatum: implications for Parkinson's disease. | Q36145938 | ||
| Dendritic cell aggresome-like induced structures are dedicated areas for ubiquitination and storage of newly synthesized defective proteins | Q36322083 | ||
| Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity. | Q36324397 | ||
| Common structure and toxic function of amyloid oligomers implies a common mechanism of pathogenesis | Q36375901 | ||
| Chaperone-mediated autophagy in aging and disease | Q36510519 | ||
| P433 | issue | 6 | |
| P921 | main subject | Huntington's disease | Q190564 |
| P577 | publication date | 2011-06-01 | |
| P1433 | published in | Cold Spring Harbor Perspectives in Biology | Q3927509 |
| P1476 | title | Huntington's Disease | |
| P478 | volume | 3 |
| Q36310758 | Allele-selective inhibition of trinucleotide repeat genes |
| Q35796020 | Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1. |
| Q62609575 | Bark Extract of the Amazonian Tree Endopleura uchi (Humiriaceae) Extends Lifespan and Enhances Stress Resistance in Caenorhabditis elegans |
| Q37292257 | CAG Expansions Are Genetically Stable and Form Nontoxic Aggregates in Cells Lacking Endogenous Polyglutamine Proteins |
| Q27305816 | Cell-based screening: extracting meaning from complex data |
| Q55498681 | Comparative Analysis of Mutant Huntingtin Binding Partners in Yeast Species. |
| Q33649261 | Copy-number variation of the neuronal glucose transporter gene SLC2A3 and age of onset in Huntington's disease |
| Q55220788 | Cranberry Extract Standardized for Proanthocyanidins Alleviates β-Amyloid Peptide Toxicity by Improving Proteostasis Through HSF-1 in Caenorhabditis elegans Model of Alzheimer's Disease. |
| Q35052612 | Development of an ELISA assay for the quantification of soluble huntingtin in human blood cells |
| Q36216005 | Fibrillar structure and charge determine the interaction of polyglutamine protein aggregates with the cell surface |
| Q89467755 | Genome-wide In Vivo CNS Screening Identifies Genes that Modify CNS Neuronal Survival and mHTT Toxicity |
| Q57021977 | Heat shock promotes inclusion body formation of mutant huntingtin (mHtt) and alleviates mHtt-induced transcription factor dysfunction |
| Q38897201 | How Do J-Proteins Get Hsp70 to Do So Many Different Things? |
| Q33852852 | Huntington's disease: from disease mechanisms to therapies |
| Q34278252 | Impaired heat shock response in cells expressing full-length polyglutamine-expanded huntingtin |
| Q38766477 | Impairment of blood-brain barrier is an early event in R6/2 mouse model of Huntington Disease |
| Q33897905 | Improved high sensitivity screen for Huntington disease using a one-step triplet-primed PCR and melting curve assay |
| Q35839766 | Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease |
| Q64076379 | Induced pluripotent stem cells from Huntington's disease patients: a promising approach to define and correct disease-related alterations |
| Q93273299 | Live-Cell Imaging and Quantification of PolyQ Aggregates by Stimulated Raman Scattering of Selective Deuterium Labeling |
| Q41525515 | Localized vs. Systematic Neurodegeneration: A Paradigm Shift in Understanding Neurodegenerative Diseases |
| Q38260329 | Mechanism and Regulation of Autophagy and Its Role in Neuronal Diseases |
| Q36477988 | Mechanism of allele-selective inhibition of huntingtin expression by duplex RNAs that target CAG repeats: function through the RNAi pathway |
| Q48023023 | Mechanisms of protein homeostasis (proteostasis) maintain stem cell identity in mammalian pluripotent stem cells |
| Q28084311 | Mediation of organismal aging and somatic proteostasis by the germline |
| Q27004209 | Modeling Huntington's disease with induced pluripotent stem cells |
| Q35048841 | Molecular interaction between the chaperone Hsc70 and the N-terminal flank of huntingtin exon 1 modulates aggregation |
| Q35921703 | Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer |
| Q26823798 | Physicochemical properties of cells and their effects on intrinsically disordered proteins (IDPs) |
| Q41183326 | Proteostasis of Huntingtin in Health and Disease |
| Q91235419 | Robust Preimplantation Genetic Testing of Huntington Disease by Combined Triplet-Primed PCR Analysis of the HTT CAG Repeat and Multi-Microsatellite Haplotyping |
| Q30355064 | Serine 421 regulates mutant huntingtin toxicity and clearance in mice. |
| Q38139218 | Single-chain fragment variable passive immunotherapies for neurodegenerative diseases |
| Q37465080 | Somatic increase of CCT8 mimics proteostasis of human pluripotent stem cells and extends C. elegans lifespan |
| Q58764761 | Studying Huntington's Disease in Yeast: From Mechanisms to Pharmacological Approaches |
| Q35137572 | The Copper Metabolism MURR1 domain protein 1 (COMMD1) modulates the aggregation of misfolded protein species in a client-specific manner |
| Q37645630 | The Mechanistic Links Between Proteasome Activity, Aging and Age-related Diseases |
| Q52738891 | The Use of Tricyclo-DNA Oligomers for the Treatment of Genetic Disorders. |
| Q52162415 | The cryo-electron microscopy structure of huntingtin |
| Q38284376 | The role of protein clearance mechanisms in organismal ageing and age-related diseases. |
| Q58414022 | The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington's disease patients |
| Q52343842 | Thermodynamics in Neurodegenerative Diseases: Interplay Between Canonical WNT/Beta-Catenin Pathway-PPAR Gamma, Energy Metabolism and Circadian Rhythms. |
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