| scholarly article | Q13442814 |
| P2093 | author name string | Yamada M | |
| Takahashi H | |||
| Wood JD | |||
| Jenkins NA | |||
| Copeland NG | |||
| Tsuji S | |||
| Margolis RL | |||
| Ross CA | |||
| Borchelt DR | |||
| Cooper JK | |||
| Duan K | |||
| Gonzales V | |||
| Price DL | |||
| Schilling G | |||
| Slunt HH | |||
| P2860 | cites work | Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity | Q22009069 |
| Atrophin-1, the DRPLA gene product, interacts with two families of WW domain-containing proteins | Q24310521 | ||
| Ataxin-3 is transported into the nucleus and associates with the nuclear matrix | Q24314312 | ||
| Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures | Q24322756 | ||
| Abnormal gene product identified in hereditary dentatorubral–pallidoluysian atrophy (DRPLA) brain | Q24324461 | ||
| A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. | Q27860836 | ||
| Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation | Q28246858 | ||
| Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p | Q28250966 | ||
| Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA) | Q28250990 | ||
| Dentatorubral pallidoluysian atrophy (DRPLA) protein is cleaved by caspase-3 during apoptosis | Q28253849 | ||
| Novel triplet repeat containing genes in human brain: cloning, expression, and length polymorphisms | Q28260268 | ||
| Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract | Q28267110 | ||
| Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo | Q28279878 | ||
| Hereditary dentatorubral-pallidoluysian atrophy: detection of widespread ubiquitinated neuronal and glial intranuclear inclusions in the brain | Q28290866 | ||
| DRPLA gene (atrophin-1) sequence and mRNA expression in human brain | Q28299557 | ||
| Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA) | Q28306588 | ||
| Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form | Q28509836 | ||
| The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1 | Q28589800 | ||
| 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 | ||
| Dentato-rubro-pallido-luysian atrophy: a clinico-pathological study | Q33620531 | ||
| 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 | ||
| Familial myoclonus epilepsy and choreoathetosis: hereditary dentatorubral-pallidoluysian atrophy | Q34270334 | ||
| Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. | Q34353488 | ||
| Unusual form of cerebellar ataxia; combined dentato-rubral and pallido-Luysian degeneration | Q34538692 | ||
| Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation | Q36255989 | ||
| Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. | Q40858681 | ||
| Caspase-8 is required for cell death induced by expanded polyglutamine repeats. | Q40961806 | ||
| Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro | Q40968069 | ||
| Trinucleotide instability: a repeating theme in human inherited disorders | Q41036331 | ||
| Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates | Q41065635 | ||
| Trinucleotide repeats in neurogenetic disorders | Q41132596 | ||
| Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract | Q41182414 | ||
| DNA analysis in hereditary dentatorubral-pallidoluysian atrophy: correlation between CAG repeat length and phenotypic variation and the molecular basis of anticipation | Q41391319 | ||
| Huntington's Disease and Dentatorubral‐Pallidoluysian Atrophy: Proteins, Pathogenesis and Pathology | Q41527116 | ||
| Dentatorubral and pallidoluysian atrophy (DRPLA). Clinical and neuropathological findings in genetically confirmed North American and European pedigrees | Q41557473 | ||
| Huntington disease and the related disorder, dentatorubral-pallidoluysian atrophy (DRPLA). | Q41628677 | ||
| Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases? | Q41680087 | ||
| 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 | ||
| Transglutaminase action imitates Huntington's disease: selective polymerization of Huntingtin containing expanded polyglutamine. | Q45296134 | ||
| 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 | ||
| Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA. | Q45296558 | ||
| Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin | Q45297223 | ||
| Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. | Q45297497 | ||
| A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. | Q45298582 | ||
| Kennedy's disease: caspase cleavage of the androgen receptor is a crucial event in cytotoxicity | Q46593765 | ||
| Intracellular aggregate formation of dentatorubral-pallidoluysian atrophy (DRPLA) protein with the extended polyglutamine | Q47680444 | ||
| Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. | Q47991885 | ||
| Identification and characterization of the gene causing type 1 spinocerebellar ataxia | Q48080540 | ||
| 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 | ||
| Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. | Q48537185 | ||
| Expression and distribution of the dentatorubral-pallidoluysian atrophy gene product (atrophin-1/drplap) in neuronal and non-neuronal tissues | Q48795581 | ||
| A vector for expressing foreign genes in the brains and hearts of transgenic mice | Q54965153 | ||
| Hereditary dentatorubral-pallidoluysian atrophy: Clinical and pathologic variants in a family | Q68420738 | ||
| Dentatorubral-pallidoluysian atrophy: clinical features are closely related to unstable expansions of trinucleotide (CAG) repeat | Q72307208 | ||
| P433 | issue | 1 | |
| P407 | language of work or name | English | Q1860 |
| P304 | page(s) | 275-286 | |
| P577 | publication date | 1999-09-01 | |
| P1433 | published in | Neuron | Q3338676 |
| P1476 | title | Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA. | |
| P478 | volume | 24 |
| Q36088535 | A mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice |
| Q40493431 | A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration. |
| Q47630786 | A novel transgenic rat model for spinocerebellar ataxia type 17 recapitulates neuropathological changes and supplies in vivo imaging biomarkers. |
| Q54965148 | Accumulation of proteolytic fragments of mutant presenilin 1 and accelerated amyloid deposition are co-regulated in transgenic mice |
| Q35097613 | Are Huntington's and polyglutamine-based ataxias proteasome storage diseases? |
| Q33207964 | Assays of nuclear localization of R7/Gbeta5 complexes |
| Q47071965 | Atrophin contributes to the negative regulation of epidermal growth factor receptor signaling in Drosophila |
| Q36980650 | Atrophin proteins: an overview of a new class of nuclear receptor corepressors |
| Q24290194 | Atrophin-1, the dentato-rubral and pallido-luysian atrophy gene product, interacts with ETO/MTG8 in the nuclear matrix and represses transcription |
| Q28589500 | C-terminal deletion of the atrophin-1 protein results in growth retardation but not neurodegeneration in mice |
| Q34460105 | Cross linking of polyglutamine domains catalyzed by tissue transglutaminase is greatly favored with pathological-length repeats: does transglutaminase activity play a role in (CAG)(n)/Q(n)-expansion diseases? |
| Q80809599 | Decreased estradiol release from astrocytes contributes to the neurodegeneration in a mouse model of Niemann-Pick disease type C |
| Q58602927 | Dentatorubral-pallidoluysian Atrophy: An Update |
| Q36539434 | Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases |
| Q39596478 | Expanded polyglutamine domain possesses nuclear export activity which modulates subcellular localization and toxicity of polyQ disease protein via exportin-1. |
| Q33916580 | Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription |
| Q91151997 | Fat cadherins in mouse models of degenerative ataxias |
| Q35639868 | Genetic and environmental factors in the pathogenesis of Huntington's disease |
| Q35058863 | Genotype-phenotype correlation in CAG-repeat diseases |
| Q90115323 | Greater Protective Potent of s-Methyl Cysteine and Syringic Acid Combination for NGF-differentiated PC12 Cells against Kainic acid-induced Injury |
| Q36599926 | Histone deacetylase inhibitors as therapeutics for polyglutamine disorders |
| Q74642989 | Huntingtin fragments that aggregate go their separate ways |
| Q28505053 | Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus |
| Q24670202 | Interaction between neuronal intranuclear inclusions and promyelocytic leukemia protein nuclear and coiled bodies in CAG repeat diseases |
| Q46463399 | Isolation of gene sets affected specifically by polyglutamine expression: implication of the TOR signaling pathway in neurodegeneration |
| Q57722118 | Isolation of neuronal cells with high processing activity for the Machado-Joseph disease protein |
| Q44185929 | Ligand promotes intranuclear inclusions in a novel cell model of spinal and bulbar muscular atrophy |
| Q35550132 | Molecular analyses of Machado-Joseph disease |
| Q34185510 | Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease |
| Q33328864 | Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration |
| Q34446518 | Molecular pathogenesis of spinal and bulbar muscular atrophy |
| Q57877560 | Mouse Models of Triplet Repeat Diseases |
| Q26853025 | Mouse models of polyglutamine diseases: review and data table. Part I |
| Q52703722 | Neurodegeneration by polyglutamine Atrophin is not rescued by induction of autophagy. |
| Q34295237 | Neuronal SUMOylation: mechanisms, physiology, and roles in neuronal dysfunction |
| Q34100009 | Neuronal nuclear alterations in dentatorubral-pallidoluysian atrophy: ultrastructural and morphometric studies of the cerebellar granule cells |
| Q45719870 | Neuroprotective effects of granulocyte-colony stimulating factor in a novel transgenic mouse model of SCA17. |
| Q42664318 | New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins |
| Q37540341 | Nuclear accumulation of polyglutamine disease proteins and neuropathology |
| Q28142336 | Nuclear localization of G protein beta 5 and regulator of G protein signaling 7 in neurons and brain |
| 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 |
| Q26796584 | Of Fighting Flies, Mice, and Men: Are Some of the Molecular and Neuronal Mechanisms of Aggression Universal in the Animal Kingdom? |
| Q48459242 | Oligodendrocytic polyglutamine pathology in dentatorubral-pallidoluysian atrophy |
| Q34185428 | Pathological accumulation of atrophin-1 in dentatorubralpallidoluysian atrophy |
| Q34158005 | Pathology of CAG repeat diseases |
| Q63951832 | Plans for HDBase—a research community website for Huntington's Disease |
| Q38210865 | Polyglutamine (PolyQ) diseases: genetics to treatments. |
| Q42651287 | Polyglutamine Atrophin provokes neurodegeneration in Drosophila by repressing fat. |
| Q36589169 | Polyglutamine disease: recent advances in the neuropathology of dentatorubral-pallidoluysian atrophy |
| Q35694039 | Polyglutamine expansion alters the dynamics and molecular architecture of aggregates in dentatorubropallidoluysian atrophy |
| Q45042684 | Polyglutamine expansion in ataxin-3 does not affect protein stability: implications for misfolding and disease. |
| Q37709833 | Polyglutamine toxicity in non-neuronal cells |
| Q37352951 | Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk |
| Q48572020 | Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice. |
| Q35949339 | Progressive myoclonic epilepsy |
| Q35608076 | Proteasome-mediated proteolysis of the polyglutamine-expanded androgen receptor is a late event in spinal and bulbar muscular atrophy (SBMA) pathogenesis |
| Q37553976 | Protein SUMOylation in neuropathological conditions. |
| Q35592405 | Protein aggregation in motor neurone disorders. |
| Q38091670 | Protein truncation as a common denominator of human neurodegenerative foldopathies |
| Q40658407 | Proteolytic cleavage and cellular toxicity of the human alpha1A calcium channel in spinocerebellar ataxia type 6. |
| Q40103515 | Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation |
| Q39639663 | Proteolytic processing regulates pathological accumulation in dentatorubral-pallidoluysian atrophy. |
| Q35575850 | Role of proteolysis in polyglutamine disorders |
| Q37090117 | Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice. |
| Q45148750 | Sharing of polyglutamine localization by the neuronal nucleus and cytoplasm in CAG-repeat diseases |
| Q46890244 | Sodium butyrate ameliorates histone hypoacetylation and neurodegenerative phenotypes in a mouse model for DRPLA. |
| Q35565116 | Spinocerebellar ataxia type 7 associated with pigmentary retinal dystrophy |
| Q47140000 | Stall in Canonical Autophagy-Lysosome Pathways Prompts Nucleophagy-Based Nuclear Breakdown in Neurodegeneration |
| Q38563516 | Studying polyglutamine diseases in Drosophila. |
| Q47799714 | Sumoylation: Implications for Neurodegenerative Diseases |
| Q37728628 | Targets and consequences of protein SUMOylation in neurons. |
| Q35808823 | The MTG proteins: chromatin repression players with a passion for networking |
| Q48351755 | The nucleus raphe interpositus in spinocerebellar ataxia type 3 (Machado-Joseph disease). |
| Q42177366 | The origin recognition core complex regulates dendrite and spine development in postmitotic neurons |
| Q36003871 | The roles of proteolysis and nuclear localisation in the toxicity of the polyglutamine diseases. A review |
| Q49019300 | Time course of polyglutamine aggregate body formation and cell death: enhanced growth in nucleus and an interval for cell death |
| Q41839150 | Transcriptional repression and cell death induced by nuclear aggregates of non-polyglutamine protein |
| Q40727142 | Ultrastructure of nuclear aggregates formed by expressing an expanded polyglutamine |
| Q33934498 | Widespread occurrence of intranuclear atrophin-1 accumulation in the central nervous system neurons of patients with dentatorubral-pallidoluysian atrophy |
| Q42765453 | Widespread white matter changes in Kennedy disease: a voxel based morphometry study |
Search more.