| scholarly article | Q13442814 |
| P6179 | Dimensions Publication ID | 1043820894 |
| P356 | DOI | 10.1038/79162 |
| P698 | PubMed publication ID | 10973246 |
| P5875 | ResearchGate publication ID | 12349380 |
| P2093 | author name string | S M Pulst | |
| K Figueroa | |||
| N Hoang | |||
| D P Huynh | |||
| P2860 | cites work | A novel protein with RNA-binding motifs interacts with ataxin-2 | Q22254111 |
| Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion | Q24310393 | ||
| Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats | Q24317096 | ||
| A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. | Q27860836 | ||
| Abundant expression and cytoplasmic aggregations of [alpha]1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6 | Q28137666 | ||
| CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1 | Q28235526 | ||
| Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation | Q28246858 | ||
| Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA) | Q28250990 | ||
| 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 | ||
| Aggresomes: a cellular response to misfolded proteins | Q28292275 | ||
| Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel | Q28300848 | ||
| The mouse SCA2 gene: cDNA sequence, alternative splicing and protein expression | Q28588597 | ||
| Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice | Q29615357 | ||
| 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 | ||
| Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy | Q34023072 | ||
| Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. | Q34353488 | ||
| Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. | Q34405223 | ||
| Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT. | Q34405231 | ||
| Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2. | Q34492842 | ||
| Lewy bodies contain altered alpha-synuclein in brains of many familial Alzheimer's disease patients with mutations in presenilin and amyloid precursor protein genes | Q35754459 | ||
| From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington's disease | Q40859661 | ||
| 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 | ||
| Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates | Q41065635 | ||
| Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold | Q41763638 | ||
| Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera | Q42870983 | ||
| The Purkinje cell and its afferents in human hereditary ataxia | Q45034738 | ||
| Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. | Q45293768 | ||
| Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo | Q45294913 | ||
| Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA. | Q45296558 | ||
| 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 | ||
| Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. | Q47991885 | ||
| Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: triple-labeling immunofluorescent study | Q48101492 | ||
| Heterogeneous intracellular localization and expression of ataxin-3. | Q48268701 | ||
| Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. | Q48298684 | ||
| Alpha synuclein is present in Lewy bodies in sporadic Parkinson's disease | Q48326512 | ||
| 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 | ||
| Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions | Q48464946 | ||
| Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. | Q48537185 | ||
| Reduced immunoreactivity to calcium-binding proteins in Purkinje cells precedes onset of ataxia in spinocerebellar ataxia-1 transgenic mice | Q48555958 | ||
| Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations. | Q48628376 | ||
| Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. | Q48644018 | ||
| Purkinje cell protein-2 regulatory regions and transgene expression in cerebellar compartments | Q48691784 | ||
| Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. | Q54066632 | ||
| SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat | Q56909286 | ||
| P433 | issue | 1 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | pathogenesis | Q372016 |
| ataxin 2 | Q21171902 | ||
| Calbindin 1 | Q21494920 | ||
| Ataxin 2 | Q21984102 | ||
| P304 | page(s) | 44-50 | |
| P577 | publication date | 2000-09-01 | |
| P1433 | published in | Nature Genetics | Q976454 |
| P1476 | title | Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human | |
| P478 | volume | 26 |
| Q24543178 | A Drosophila homolog of the polyglutamine disease gene SCA2 is a dosage-sensitive regulator of actin filament formation |
| Q39732144 | A Drosophila model of Huntington disease-like 2 exhibits nuclear toxicity and distinct pathogenic mechanisms from Huntington disease |
| Q40144785 | A cell-based model of alpha-synucleinopathy for screening compounds with therapeutic potential of Parkinson's disease |
| Q37852582 | A comprehensive review of spinocerebellar ataxia type 2 in Cuba |
| Q36231685 | A polyglutamine expansion disease protein sequesters PTIP to attenuate DNA repair and increase genomic instability |
| Q48474780 | A randomized controlled pilot trial of lithium in spinocerebellar ataxia type 2. |
| Q39319522 | ALS-associated ataxin 2 polyQ expansions enhance stress-induced caspase 3 activation and increase TDP-43 pathological modifications |
| Q34391155 | ATXN2 and its neighbouring gene SH2B3 are associated with increased ALS risk in the Turkish population |
| Q48045147 | ATXN2-AS, a gene antisense to ATXN2, is associated with spinocerebellar ataxia type 2 and amyotrophic lateral sclerosis. |
| Q34407813 | ATXN2-CAG42 sequesters PABPC1 into insolubility and induces FBXW8 in cerebellum of old ataxic knock-in mice. |
| Q57970917 | Age at onset variance analysis in spinocerebellar ataxias: A study in a Dutch-French cohort |
| Q24338666 | An integrative approach to gain insights into the cellular function of human ataxin-2 |
| Q84383056 | Animal models of human cerebellar ataxias: a cornerstone for the therapies of the twenty-first century |
| Q24655528 | Animal models of polyglutamine diseases and therapeutic approaches |
| Q45984659 | Antisense oligonucleotide therapy for spinocerebellar ataxia type 2. |
| Q41098241 | Ataxin-2 (Atxn2)-Knock-Out Mice Show Branched Chain Amino Acids and Fatty Acids Pathway Alterations |
| Q24317006 | Ataxin-2 and huntingtin interact with endophilin-A complexes to function in plastin-associated pathways |
| Q42054445 | Ataxin-2 mediated cell death is dependent on domains downstream of the polyQ repeat |
| Q34172531 | Ataxin-2 promotes apoptosis of human neuroblastoma cells |
| Q27311213 | Ataxin-2 regulates RGS8 translation in a new BAC-SCA2 transgenic mouse model |
| Q33836795 | Ataxin-2: From RNA Control to Human Health and Disease |
| Q35196519 | Both ubiquitin ligases FBXW8 and PARK2 are sequestrated into insolubility by ATXN2 PolyQ expansions, but only FBXW8 expression is dysregulated |
| Q24314234 | C-termini of P/Q-type Ca2+ channel alpha1A subunits translocate to nuclei and promote polyglutamine-mediated toxicity |
| Q36477656 | Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2 |
| Q37716781 | Chaperones in Polyglutamine Aggregation: Beyond the Q-Stretch. |
| Q36315976 | Chronic suppression of inositol 1,4,5-triphosphate receptor-mediated calcium signaling in cerebellar purkinje cells alleviates pathological phenotype in spinocerebellar ataxia 2 mice |
| Q64066929 | Circadian control of stress granules by oscillating EIF2α |
| Q34992022 | Computational neurobiology is a useful tool in translational neurology: the example of ataxia |
| Q27028008 | Current understanding of the role of microRNAs in spinocerebellar ataxias |
| Q37008921 | Degenerative ataxias, from genes to therapies: The 2015 Cotzias Lecture |
| Q37359666 | Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2. |
| Q35670193 | Deranged calcium signaling in Purkinje cells and pathogenesis in spinocerebellar ataxia 2 (SCA2) and other ataxias |
| Q38792714 | Discovery of Therapeutic Approaches for Polyglutamine Diseases: A Summary of Recent Efforts |
| Q92354437 | Disrupted Calcium Signaling in Animal Models of Human Spinocerebellar Ataxia (SCA) |
| Q84040853 | Distribution and pattern of pathology in subjects with familial or sporadic late-onset cerebellar ataxia as assessed by p62/sequestosome immunohistochemistry |
| Q38408049 | Disturbed calcium signaling in spinocerebellar ataxias and Alzheimer's disease |
| Q34390181 | ETS1 regulates the expression of ATXN2. |
| Q43963278 | Expanded polyglutamine stretches form an 'aggresome'. |
| Q28589064 | Expression of PTPRO in the interneurons of adult mouse olfactory bulb |
| Q36742104 | Expression of expanded polyglutamine targets profilin for degradation and alters actin dynamics |
| Q48965275 | Extended pathoanatomical studies point to a consistent affection of the thalamus in spinocerebellar ataxia type 2. |
| Q38578808 | From mice to men: lessons from mutant ataxic mice |
| Q38259591 | From pathways to targets: understanding the mechanisms behind polyglutamine disease. |
| Q51931311 | Frontotemporal cognitive function in X-linked spinal and bulbar muscular atrophy (SBMA): a controlled neuropsychological study of 20 patients. |
| Q36717931 | Generation of human-induced pluripotent stem cells to model spinocerebellar ataxia type 2 in vitro |
| Q38300785 | Genetic ablation of ataxin-2 increases several global translation factors in their transcript abundance but decreases translation rate |
| Q37196137 | Genetic and molecular aspects of spinocerebellar ataxias |
| Q33519049 | Genetic variance in the spinocerebellar ataxia type 2 (ATXN2) gene in children with severe early onset obesity |
| Q37910368 | Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias |
| Q73174347 | Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein |
| Q44320440 | Histone H3 is aberrantly phosphorylated in glutamine-repeat diseases |
| Q44569570 | Hprt(CAG)146 mice: age of onset of behavioral abnormalities, time course of neuronal intranuclear inclusion accumulation, neurotransmitter marker alterations, mitochondrial function markers, and susceptibility to 1-methyl-4-phenyl-1,2,3,6-tetrahydro |
| Q34797845 | Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases |
| Q30892767 | Identification and expression of the gene for human ataxin-2-related protein on chromosome 16. |
| Q48237961 | Immunohistochemical characterization of substance P receptor (NK(1)R)-expressing interneurons in the entorhinal cortex. |
| Q89017844 | In Vivo Analysis of the Climbing Fiber-Purkinje Cell Circuit in SCA2-58Q Transgenic Mouse Model |
| Q37042016 | In vivo analysis of cerebellar Purkinje cell activity in SCA2 transgenic mouse model |
| Q37201299 | Inositol 1,4,5-tripshosphate receptor, calcium signaling, and polyglutamine expansion disorders |
| Q49666213 | Inositol 1,4,5-trisphosphate receptors and neurodegenerative disorders |
| Q35550098 | Insights into the molecular basis of polyglutamine neurodegeneration from studies of a spinocerebellar ataxia type 7 mouse model |
| Q27301865 | Interaction of RNA with a C-terminal fragment of the amyotrophic lateral sclerosis-associated TDP43 reduces cytotoxicity |
| Q38168374 | Intracellular calcium channels: inositol-1,4,5-trisphosphate receptors |
| Q38667591 | Linking Essential Tremor to the Cerebellum-Animal Model Evidence |
| Q48706252 | Localization of ataxin-2 within the cerebellar cortex of the rat. |
| Q35215622 | Massive expansion of SCA2 with autonomic dysfunction, retinitis pigmentosa, and infantile spasms |
| Q64969037 | Mechanisms of protein toxicity in neurodegenerative diseases. |
| Q42599213 | Meeting report of the European mouse complex genetics network SYSGENET. |
| Q35217890 | Mesenchymal stem cell transplantation ameliorates motor function deterioration of spinocerebellar ataxia by rescuing cerebellar Purkinje cells |
| Q40473493 | Misfolding promotes the ubiquitination of polyglutamine-expanded ataxin-3, the defective gene product in SCA3/MJD. |
| Q61800987 | Modeling spinocerebellar ataxias 2 and 3 with iPSCs reveals a role for glutamate in disease pathology |
| Q38818474 | Modulation of Molecular Chaperones in Huntington's Disease and Other Polyglutamine Disorders. |
| Q35087285 | Molecular mechanism of Spinocerebellar Ataxia type 6: glutamine repeat disorder, channelopathy and transcriptional dysregulation. The multifaceted aspects of a single mutation. |
| Q36774733 | Molecular pathogenesis of spinocerebellar ataxia type 6. |
| Q28076174 | Motor Dysfunctions and Neuropathology in Mouse Models of Spinocerebellar Ataxia Type 2: A Comprehensive Review |
| Q57877560 | Mouse Models of Triplet Repeat Diseases |
| Q36358879 | Mouse models of Machado-Joseph disease and other polyglutamine spinocerebellar ataxias |
| Q26853025 | Mouse models of polyglutamine diseases: review and data table. Part I |
| Q58786956 | Neonatal brain injury causes cerebellar learning deficits and Purkinje cell dysfunction |
| Q35560289 | Neurogenetics: single gene disorders |
| Q44607270 | Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport |
| Q45719870 | Neuroprotective effects of granulocyte-colony stimulating factor in a novel transgenic mouse model of SCA17. |
| Q28585964 | Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence |
| Q42585068 | Nucleation of protein aggregation kinetics as a basis for genotype-phenotype correlations in polyglutamine diseases. |
| Q33463406 | Nucleocytoplasmic shuttling activity of ataxin-3. |
| Q33791247 | Oligonucleotide-based strategies to combat polyglutamine diseases |
| Q38008054 | Oxidative stress as a cofactor in spinocerebellar ataxia type 2. |
| Q34446581 | PQBP-1 (Np/PQ): a polyglutamine tract-binding and nuclear inclusion-forming protein |
| Q24313820 | Parkin is an E3 ubiquitin-ligase for normal and mutant ataxin-2 and prevents ataxin-2-induced cell death |
| Q48748406 | Pathoanatomy of cerebellar degeneration in spinocerebellar ataxia type 2 (SCA2) and type 3 (SCA3). |
| Q35841041 | Pathways to motor incoordination: the inherited ataxias |
| Q47631159 | Peripheral markers of autophagy in polyglutamine diseases. |
| Q33319615 | Polyglutamine genes interact to modulate the severity and progression of neurodegeneration in Drosophila |
| Q47880472 | Polyglutamine spinocerebellar ataxias - from genes to potential treatments |
| Q28189688 | Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7 |
| Q35150291 | Polyglutamines placed into context |
| Q39092630 | Prion-Like Characteristics of Polyglutamine-Containing Proteins |
| Q39159540 | Prodromal spinocerebellar ataxia type 2: Prospects for early interventions and ethical challenges |
| Q43792416 | Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease. |
| Q45735441 | Protein Misfolding and Aggregation as a Therapeutic Target for Polyglutamine Diseases |
| Q35110875 | RNA editing in Drosophila melanogaster: New targets and functional consequences |
| Q35789502 | RNA-binding protein misregulation in microsatellite expansion disorders |
| Q37155206 | RNA-binding proteins in neurodegenerative disease: TDP-43 and beyond |
| Q35576413 | Recent advances in understanding the pathogenesis of polyglutamine diseases: involvement of molecular chaperones and ubiquitin-proteasome pathway. |
| Q35667019 | Repeat Associated Non-AUG Translation (RAN Translation) Dependent on Sequence Downstream of the ATXN2 CAG Repeat |
| Q33894384 | Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment. |
| Q34981549 | Role of inositol 1,4,5-trisphosphate receptors in pathogenesis of Huntington's disease and spinocerebellar ataxias |
| Q35575850 | Role of proteolysis in polyglutamine disorders |
| Q30431384 | Roles for gamma-aminobutyric acid in the development of the paraventricular nucleus of the hypothalamus |
| Q36354944 | Selective positive modulator of calcium-activated potassium channels exerts beneficial effects in a mouse model of spinocerebellar ataxia type 2 |
| Q45148750 | Sharing of polyglutamine localization by the neuronal nucleus and cytoplasm in CAG-repeat diseases |
| Q93041355 | Spinocerebellar ataxia |
| Q37140787 | Spinocerebellar ataxia 2 (SCA2). |
| Q48671892 | Spinocerebellar ataxia type 1 (SCA1): new pathoanatomical and clinico-pathological insights |
| Q38045212 | Spinocerebellar ataxia type 2: clinical presentation, molecular mechanisms, and therapeutic perspectives |
| Q40407727 | Spinocerebellar ataxia type 2: polyQ repeat variation in the CACNA1A calcium channel modifies age of onset |
| Q48957430 | Spinocerebellar ataxias types 2 and 3: degeneration of the pre-cerebellar nuclei isolates the three phylogenetically defined regions of the cerebellum |
| Q44604174 | Spinocerebellar degeneration |
| Q58759815 | Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration |
| Q41510206 | The Multiple Faces of Spinocerebellar Ataxia type 2. |
| Q60306591 | The complex structure of genetic variation |
| Q48351755 | The nucleus raphe interpositus in spinocerebellar ataxia type 3 (Machado-Joseph disease). |
| Q35340099 | The role for alterations in neuronal activity in the pathogenesis of polyglutamine repeat disorders |
| Q36003871 | The roles of proteolysis and nuclear localisation in the toxicity of the polyglutamine diseases. A review |
| Q29619760 | The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans |
| Q30410969 | Therapeutic prospects for spinocerebellar ataxia type 2 and 3. |
| Q49019300 | Time course of polyglutamine aggregate body formation and cell death: enhanced growth in nucleus and an interval for cell death |
| Q34446513 | Triplet repeats, RNA secondary structure and toxic gain-of-function models for pathogenesis |
| Q27314605 | dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1 |
| Q24647507 | miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis |
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