| scholarly article | Q13442814 |
| P50 | author | Jillian K. Cooper | Q73662802 |
| P2093 | author name string | A H Sharp | |
| C A Ross | |||
| M F Peters | |||
| D R Borchelt | |||
| T M Dawson | |||
| V L Dawson | |||
| F A Khan | |||
| G Schilling | |||
| M Delanoy | |||
| W J Herring | |||
| Z Kaminsky | |||
| J Masone | |||
| P433 | issue | 5 | |
| P407 | language of work or name | English | Q1860 |
| P304 | page(s) | 783-790 | |
| P577 | publication date | 1998-05-01 | |
| P1433 | published in | Human Molecular Genetics | Q2720965 |
| P1476 | title | Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture | |
| P478 | volume | 7 |
| Q45298582 | A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. |
| Q38174451 | A mitochondrial basis for Huntington's disease: therapeutic prospects |
| Q35430697 | A molecular investigation of true dominance in Huntington's disease |
| Q24683342 | A role for tissue transglutaminase in alpha-gliadin peptide cytotoxicity |
| Q39832548 | ATF3 plays a protective role against toxicity by N-terminal fragment of mutant huntingtin in stable PC12 cell line |
| Q38720406 | Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation |
| Q37142133 | Acetylation within the First 17 Residues of Huntingtin Exon 1 Alters Aggregation and Lipid Binding. |
| Q35103285 | Activated microglia proliferate at neurites of mutant huntingtin-expressing neurons |
| Q45288978 | Adenovirus vector-based in vitro neuronal cell model for Huntington's disease with human disease-like differential aggregation and degeneration |
| Q41513084 | Aggregation behavior of chemically synthesized, full-length huntingtin exon1. |
| Q37238530 | Aggregation formation in the polyglutamine diseases: protection at a cost? |
| Q43100873 | Aggregation landscapes of Huntingtin exon 1 protein fragments and the critical repeat length for the onset of Huntington's disease |
| Q41920050 | Alpha-synuclein overexpression promotes aggregation of mutant huntingtin |
| Q33949223 | Altered transcription in yeast expressing expanded polyglutamine |
| Q28731292 | Amyloid-like fibril formation by polyQ proteins: a critical balance between the polyQ length and the constraints imposed by the host protein |
| Q37198517 | An Intrabody Drug (rAAV6-INT41) Reduces the Binding of N-Terminal Huntingtin Fragment(s) to DNA to Basal Levels in PC12 Cells and Delays Cognitive Loss in the R6/2 Animal Model |
| Q34510480 | Analysis of proteolytic processes and enzymatic activities in the generation of huntingtin n-terminal fragments in an HEK293 cell model. |
| Q73212216 | Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease |
| Q34605368 | Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophy |
| Q40641130 | Antisense downregulation of mutant huntingtin in a cell model. |
| Q35092246 | Apoptosis in Huntington's disease. |
| Q35097613 | Are Huntington's and polyglutamine-based ataxias proteasome storage diseases? |
| Q33703905 | Are there multiple pathways in the pathogenesis of Huntington's disease? |
| Q50864652 | Assembly of Huntingtin headpiece into α-helical bundles. |
| Q24316293 | Ataxin 1, a SCA1 neurodegenerative disorder protein, is functionally linked to the silencing mediator of retinoid and thyroid hormone receptors |
| Q35212735 | Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease |
| Q33675599 | Biophysical underpinnings of the repeat length dependence of polyglutamine amyloid formation |
| Q35906784 | Calcium Handling by Endoplasmic Reticulum and Mitochondria in a Cell Model of Huntington's Disease. |
| Q40704833 | Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. |
| Q33873247 | Caspases and neurodegeneration: on the cutting edge of new therapeutic approaches. |
| Q34476762 | Caspases in Huntington's disease |
| Q90140618 | Cell-Autonomous and Non-cell-Autonomous Pathogenic Mechanisms in Huntington's Disease: Insights from In Vitro and In Vivo Models |
| Q77906721 | Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin |
| Q34142252 | Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases |
| Q40773928 | Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation |
| Q44865087 | Characterization of intracellular aggregates using fluorescently-tagged polyglutamine-expanded androgen receptor |
| Q22009069 | Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity |
| Q35105749 | Clinical and Research Advances in Huntington's Disease |
| Q34752467 | Cysteine proteases bleomycin hydrolase and cathepsin Z mediate N-terminal proteolysis and toxicity of mutant huntingtin |
| Q33931283 | Differential vulnerability of neurons in Huntington's disease: the role of cell type-specific features |
| Q35239346 | Disruption of the nuclear membrane by perinuclear inclusions of mutant huntingtin causes cell-cycle re-entry and striatal cell death in mouse and cell models of Huntington's disease. |
| Q37723743 | Dysregulation of intracellular dopamine stores revealed in the R6/2 mouse striatum |
| Q35084195 | Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease |
| Q33894812 | Effects of intracellular expression of anti-huntingtin antibodies of various specificities on mutant huntingtin aggregation and toxicity |
| Q34505628 | Electroconvulsive shock ameliorates disease processes and extends survival in huntingtin mutant mice. |
| Q36150628 | Emerging chemotherapeutic strategies for Huntington's disease |
| Q33703919 | Evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease in cell culture and in transgenic mice expressing mutant huntingtin. |
| Q58041483 | Excitotoxicity in Huntington disease |
| Q47378548 | Expression of the hyperphosphorylated tau attenuates ER stress-induced apoptosis with upregulation of unfolded protein response |
| Q34138249 | Familial conformational diseases and dementias |
| Q33374464 | Focal distortion of the nuclear envelope by huntingtin aggregates revealed by lamin immunostaining |
| Q33911387 | Formation of morphologically similar globular aggregates from diverse aggregation-prone proteins in mammalian cells |
| Q45298039 | Formation of polyglutamine inclusions in non-CNS tissue |
| Q36861129 | Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease. |
| Q28592352 | HAP1 facilitates effects of mutant huntingtin on inositol 1,4,5-trisphosphate-induced Ca release in primary culture of striatal medium spiny neurons |
| Q48373564 | Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. |
| Q24310249 | Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1 |
| Q36324397 | Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity. |
| Q34328850 | Huntingtin fragments and SOD1 mutants form soluble oligomers in the cell |
| Q40717667 | Huntingtin is required for ciliogenesis and neurogenesis during early Xenopus development |
| Q57839949 | Huntingtin proteolysis in Huntington disease |
| Q98471370 | Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities |
| Q45306758 | Huntington's disease and mitochondrial DNA deletions: event or regular mechanism for mutant huntingtin protein and CAG repeats expansion?! |
| Q37889820 | Huntington's disease, calcium, and mitochondria |
| Q35038660 | Huntington's disease: the mystery unfolds? |
| Q37424600 | Hyperactivity and cortical disinhibition in mice with restricted expression of mutant huntingtin to parvalbumin-positive cells |
| Q36159463 | Impaired GAPDH-induced mitophagy contributes to the pathology of Huntington's disease |
| Q43925468 | Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease |
| Q30779186 | Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells |
| Q41831936 | Intracellular green fluorescent protein-polyalanine aggregates are associated with cell death |
| Q33607537 | Intracellular inclusions, pathological markers in diseases caused by expanded polyglutamine tracts? |
| Q40859984 | Intracellular processing and toxicity of the truncated androgen receptor: nuclear congophilia-associated cell death |
| Q45289195 | Investigation of tRNA(Leu/Lys) and ATPase 6 genes mutations in Huntington's disease |
| Q40644113 | Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length. |
| Q45296620 | Luminescent platforms for monitoring changes in the solubility of amylin and huntingtin in living cells |
| Q34408222 | Modeling Huntington's disease in cells, flies, and mice |
| Q40556729 | Modulating huntingtin half-life alters polyglutamine-dependent aggregate formation and cell toxicity |
| Q37776087 | Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease |
| Q34443867 | Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity |
| Q34185510 | Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease |
| Q36774733 | Molecular pathogenesis of spinocerebellar ataxia type 6. |
| Q33788913 | Monomeric, oligomeric and polymeric proteins in huntington disease and other diseases of polyglutamine expansion |
| Q39885698 | Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells |
| Q43953876 | Mutant huntingtin aggregates do not sensitize cells to apoptotic stressors |
| Q48298684 | Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. |
| Q40774768 | Mutant protein in Huntington disease is resistant to proteolysis in affected brain |
| Q46661055 | N-Terminal Fragments of Huntingtin Longer than Residue 170 form Visible Aggregates Independently to Polyglutamine Expansion. |
| Q37294750 | Neocortical expression of mutant huntingtin is not required for alterations in striatal gene expression or motor dysfunction in a transgenic mouse |
| Q44535393 | Neocortical neurons cultured from mice with expanded CAG repeats in the huntingtin gene: unaltered vulnerability to excitotoxins and other insults |
| Q28587499 | Neuronal degeneration in the basal ganglia and loss of pallido-subthalamic synapses in mice with targeted disruption of the Huntington's disease gene |
| Q34208531 | Nitric oxide and nitric oxide synthase in Huntington's disease |
| Q34514433 | No post-genetics era in human disease research |
| Q37189443 | Non-targeted identification of prions and amyloid-forming proteins from yeast and mammalian cells |
| Q33890266 | Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA. |
| Q45297497 | Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. |
| Q33950288 | Nuclear localization and dominant-negative suppression by a mutant SKCa3 N-terminal channel fragment identified in a patient with schizophrenia. |
| Q44095469 | Nuclear localization of N-terminal mutant huntingtin is cell cycle dependent |
| Q28593520 | Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity |
| Q28585964 | Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence |
| Q73965077 | Nuclear relocation of normal huntingtin |
| Q35896064 | Nuclear retention of full-length HTT RNA is mediated by splicing factors MBNL1 and U2AF65 |
| Q45290815 | Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat. |
| Q43207028 | PRMT5- mediated symmetric arginine dimethylation is attenuated by mutant huntingtin and is impaired in Huntington's disease (HD). |
| Q48291079 | Pathogenesis of neurodegenerative diseases associated with expanded glutamine repeats: new answers, new questions. |
| Q27007098 | Pathogenic mechanisms and therapeutic strategies in spinobulbar muscular atrophy |
| Q35652167 | Phosphorylation of tau antagonizes apoptosis by stabilizing beta-catenin, a mechanism involved in Alzheimer's neurodegeneration |
| Q29614494 | Plasma membrane contributes to the formation of pre-autophagosomal structures |
| Q38261844 | Polyglutamine Aggregation in Huntington Disease: Does Structure Determine Toxicity? |
| Q33703910 | Polyglutamine pathogenesis. |
| Q37352951 | Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk |
| Q45296540 | Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. |
| Q34797015 | Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron |
| Q38806392 | Potential applications of stress solutes from extremophiles in protein folding diseases and healthcare |
| Q37812676 | Potential of cystamine and cysteamine in the treatment of neurodegenerative diseases |
| Q30499096 | Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment. |
| Q45299414 | Preparation of human cDNas encoding expanded polyglutamine repeats |
| Q38166717 | Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins |
| Q33703914 | Properties of polyglutamine expansion in vitro and in a cellular model for Huntington's disease. |
| Q34498308 | Protein aggregates and dementia: is there a common toxicity? |
| Q34081469 | Protein aggregation and pathogenesis of Huntington's disease: mechanisms and correlations |
| Q41763638 | Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold |
| Q45296843 | Protein precipitation: a common etiology in neurodegenerative disorders? |
| Q38091670 | Protein truncation as a common denominator of human neurodegenerative foldopathies |
| Q44133868 | Protein tyrosine phosphatases are up-regulated and participate in cell death induced by polyglutamine expansion |
| Q38855572 | Quercetin improves the activity of the ubiquitin-proteasomal system in 150Q mutated huntingtin-expressing cells but exerts detrimental effects on neuronal survivability |
| Q45306743 | R6/2 neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric mice |
| Q55414200 | RNA Aptamers Rescue Mitochondrial Dysfunction in a Yeast Model of Huntington’s Disease. |
| Q33650539 | Recent advances in understanding the pathogenesis of Huntington's disease |
| Q36255989 | Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation |
| Q45297539 | Riluzole therapy in Huntington's disease (HD). |
| Q35575850 | Role of proteolysis in polyglutamine disorders |
| Q34646828 | Side-chain interactions determine amyloid formation by model polyglutamine peptides in molecular dynamics simulations |
| Q33949081 | Specificity in intracellular protein aggregation and inclusion body formation |
| Q64261990 | Structural and dynamic studies reveal that the Ala-rich region of ataxin-7 initiates α-helix formation of the polyQ tract but suppresses its aggregation |
| Q46559063 | Study of the aggregation mechanism of polyglutamine peptides using replica exchange molecular dynamics simulations |
| Q38165760 | Studying neurodegenerative diseases in culture models. |
| Q40958511 | Subtype-specific enhancement of NMDA receptor currents by mutant huntingtin |
| Q36448271 | Targeting expression of expanded polyglutamine proteins to the endoplasmic reticulum or mitochondria prevents their aggregation. |
| Q28728849 | Temporal separation of aggregation and ubiquitination during early inclusion formation in transgenic mice carrying the Huntington's disease mutation |
| Q24338933 | The DNAJB6 and DNAJB8 protein chaperones prevent intracellular aggregation of polyglutamine peptides |
| Q50551501 | The composition of the polyglutamine-containing proteins influences their co-aggregation properties. |
| Q34019143 | The early cellular pathology of Huntington's disease |
| Q36110838 | The emerging role of the first 17 amino acids of huntingtin in Huntington's disease |
| Q73756726 | The fate of the nuclear matrix-associated-region-binding protein SATB1 during apoptosis |
| Q41035353 | The influence of huntingtin protein size on nuclear localization and cellular toxicity. |
| Q36521932 | The role of mitochondria in inherited neurodegenerative diseases |
| Q34216757 | The selective vulnerability of nerve cells in Huntington's disease |
| Q37008236 | The structural impact of a polyglutamine tract is location-dependent |
| Q28587110 | Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats |
| Q36342395 | Tissue transglutaminase does not contribute to the formation of mutant huntingtin aggregates |
| Q33891342 | Toward an understanding of polyglutamine neurodegeneration |
| Q34001953 | Transcriptional dysregulation in Huntington's disease |
| Q48357496 | Transgenic mice in the study of polyglutamine repeat expansion diseases |
| Q57747382 | Triggering of neuronal cell death by accumulation of activated SEK1 on nuclear polyglutamine aggregations in PML bodies |
| Q35633648 | Triplet repeat RNA structure and its role as pathogenic agent and therapeutic target |
| Q40880907 | Wild-type huntingtin protects from apoptosis upstream of caspase-3. |
| Q34044164 | Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo |
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