scholarly article | Q13442814 |
P819 | ADS bibcode | 1999PNAS...96..179F |
P356 | DOI | 10.1073/PNAS.96.1.179 |
P932 | PMC publication ID | 15113 |
P698 | PubMed publication ID | 9874792 |
P5875 | ResearchGate publication ID | 13413248 |
P2093 | author name string | A C Hart | |
J R Alter | |||
M E MacDonald | |||
P W Faber | |||
P2860 | cites work | 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 | ||
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 | ||
Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo | Q28279878 | ||
Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel | Q28300848 | ||
Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion | Q28594526 | ||
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice | Q29615357 | ||
Mutant sensory cilia in the nematode Caenorhabditis elegans | Q29615728 | ||
DNA transformation | Q29616766 | ||
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain | Q29617982 | ||
Mutations affecting the chemosensory neurons of Caenorhabditis elegans | Q33964180 | ||
Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy | Q34023072 | ||
Axonal guidance mutants of Caenorhabditis elegans identified by filling sensory neurons with fluorescein dyes | Q34195540 | ||
Divergent seven transmembrane receptors are candidate chemosensory receptors in C. elegans. | Q34297493 | ||
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 | ||
A dual mechanosensory and chemosensory neuron in Caenorhabditis elegans | Q36161991 | ||
Normal and expanded Huntington's disease gene alleles produce distinguishable proteins due to translation across the CAG repeat. | Q36437616 | ||
Chemosensory cell function in the behavior and development of Caenorhabditis elegans | Q37843934 | ||
Huntington's disease. Pathogenesis and management | Q39460676 | ||
Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. | Q40858681 | ||
Huntington's disease: CAG genetics expands neurobiology | Q40958023 | ||
The influence of huntingtin protein size on nuclear localization and cellular toxicity. | Q41035353 | ||
Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats | Q41041541 | ||
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates | Q41065635 | ||
Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias | Q41269608 | ||
The complex pathology of trinucleotide repeats | Q41477080 | ||
In situ evidence for DNA fragmentation in Huntingtonʼs disease striatum and Alzheimerʼs disease temporal lobes | Q41678711 | ||
Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases? | Q41680087 | ||
Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues | Q42503942 | ||
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo | Q45294913 | ||
Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. | Q45296540 | ||
Neuropathological classification of Huntington's disease | Q45297167 | ||
Caenorhabditis elegans rab-3 mutant synapses exhibit impaired function and are partially depleted of vesicles. | Q46113273 | ||
Analysis of dominant-negative mutations of the Caenorhabditis elegans let-60 ras gene | Q46791219 | ||
A modular set of lacZ fusion vectors for studying gene expression in Caenorhabditis elegans | Q47938991 | ||
Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila | Q47945602 | ||
Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse | Q48574817 | ||
SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat | Q56909286 | ||
Programmed cell death in Caenorhabditis elegans | Q57939597 | ||
Synaptic code for sensory modalities revealed by C. elegans GLR-1 glutamate receptor | Q59062681 | ||
P433 | issue | 1 | |
P407 | language of work or name | English | Q1860 |
P921 | main subject | apoptotic process | Q14599311 |
Caenorhabditis elegans | Q91703 | ||
P1104 | number of pages | 6 | |
P304 | page(s) | 179-184 | |
P577 | publication date | 1999-01-01 | |
P1433 | published in | Proceedings of the National Academy of Sciences of the United States of America | Q1146531 |
P1476 | title | Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron | |
P478 | volume | 96 |
Q41958028 | A C. elegans homolog of huntingtin-associated protein 1 is expressed in chemosensory neurons and in a number of other somatic cell types |
Q54960388 | A Liquid to Solid Phase Transition Underlying Pathological Huntingtin Exon1 Aggregation. |
Q45303122 | A Nematode Nobel Prize: Caenorhabditis elegans |
Q36246438 | A bacterial metabolite induces glutathione-tractable proteostatic damage, proteasomal disturbances, and PINK1-dependent autophagy in C. elegans |
Q27335238 | A genetic screening strategy identifies novel regulators of the proteostasis network |
Q38974551 | A link between chromatin condensation mechanisms and Huntington's disease: connecting the dots. |
Q36304420 | A new Caenorhabditis elegans model of human huntingtin 513 aggregation and toxicity in body wall muscles |
Q36779433 | A protocol describing pharynx counts and a review of other assays of apoptotic cell death in the nematode worm Caenorhabditis elegans |
Q33816233 | ASIP Outstanding Investigator Award Lecture. New approaches to the pathology and genetics of neurodegeneration |
Q41773329 | Acetylation targets mutant huntingtin to autophagosomes for degradation |
Q55405670 | Adeno-Associated Viral Vector Serotype DJ-Mediated Overexpression of N171-82Q-Mutant Huntingtin in the Striatum of Juvenile Mice Is a New Model for Huntington's Disease. |
Q33633045 | Aggregates in neurodegenerative disease: crowds and power? |
Q41523783 | Aggregation-prone GFAP mutation in Alexander disease validated using a zebrafish model. |
Q33949223 | Altered transcription in yeast expressing expanded polyglutamine |
Q26852143 | Alternatives to animal testing: A review |
Q36606399 | Animal models of Huntington's disease: implications in uncovering pathogenic mechanisms and developing therapies |
Q38165759 | Animal models of neurodegenerative diseases |
Q35092246 | Apoptosis in Huntington's disease. |
Q41664405 | Approaches for Studying Autophagy in Caenorhabditis elegans |
Q21562132 | Automated high-content live animal drug screening using C. elegans expressing the aggregation prone serpin α1-antitrypsin Z |
Q35751839 | Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis |
Q37114262 | Behavioral analysis of the huntingtin-associated protein 1 ortholog trak-1 in Caenorhabditis elegans |
Q27325393 | C. elegans expressing human β2-microglobulin: a novel model for studying the relationship between the molecular assembly and the toxic phenotype |
Q37438482 | Caenorhabditis elegans as a model system for studying non-cell-autonomous mechanisms in protein-misfolding diseases |
Q33658107 | Caenorhabditis elegans: a model to investigate oxidative stress and metal dyshomeostasis in Parkinson's disease |
Q36927018 | Caenorhabditis elegans: an emerging model in biomedical and environmental toxicology. |
Q39222757 | Candida albicans Is Resistant to Polyglutamine Aggregation and Toxicity |
Q24554218 | Cdc42-interacting protein 4 binds to huntingtin: neuropathologic and biological evidence for a role in Huntington's disease |
Q34142252 | Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases |
Q36194355 | Chaperone networks: tipping the balance in protein folding diseases |
Q38139868 | Choosing an animal model for the study of Huntington's disease |
Q44088939 | Circumvention of chaperone requirement for aggregate formation of a short polyglutamine tract by the co-expression of a long polyglutamine tract |
Q35105749 | Clinical and Research Advances in Huntington's Disease |
Q30496117 | Coenzyme Q protects Caenorhabditis elegans GABA neurons from calcium-dependent degeneration |
Q55498681 | Comparative Analysis of Mutant Huntingtin Binding Partners in Yeast Species. |
Q50749960 | Conversion of green fluorescent protein into a toxic, aggregation-prone protein by C-terminal addition of a short peptide. |
Q35644847 | Dictyostelium discoideum has a highly Q/N-rich proteome and shows an unusual resilience to protein aggregation. |
Q30488783 | Dietary restriction suppresses proteotoxicity and enhances longevity by an hsf-1-dependent mechanism in Caenorhabditis elegans |
Q45300254 | Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity. |
Q34804747 | Disrupted-in-Schizophrenia 1-mediated axon guidance involves TRIO-RAC-PAK small GTPase pathway signaling |
Q38574794 | Drosophila as a model to study the role of glia in neurodegeneration |
Q35074847 | Dying for a cause: invertebrate genetics takes on human neurodegeneration |
Q64237552 | Effects of Thioflavin T and GSK-3 Inhibition on Lifespan and Motility in a Model of Tauopathy |
Q33949349 | Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death |
Q38108756 | Experimental models for identifying modifiers of polyglutamine-induced aggregation and neurodegeneration |
Q49168922 | Finding function in novel targets: C. elegans as a model organism |
Q34079552 | Fluoxetine protects against amyloid-beta toxicity, in part via daf-16 mediated cell signaling pathway, in Caenorhabditis elegans |
Q35764318 | Genetic and pharmacological suppression of polyglutamine-dependent neuronal dysfunction in Caenorhabditis elegans |
Q52651349 | Glial and neuronal expression of polyglutamine proteins induce behavioral changes and aggregate formation in Drosophila. |
Q34431070 | Glutamine/proline-rich PQE-1 proteins protect Caenorhabditis elegans neurons from huntingtin polyglutamine neurotoxicity |
Q44155992 | Golgi fragmentation occurs in the cells with prefibrillar alpha-synuclein aggregates and precedes the formation of fibrillar inclusion |
Q36834709 | Heat shock genes - integrating cell survival and death |
Q45298333 | History of genetic disease: the molecular genetics of Huntington disease - a history |
Q34858502 | Human disease models in Drosophila melanogaster and the role of the fly in therapeutic drug discovery |
Q24554374 | Huntingtin in health and disease |
Q83728053 | Huntington's Disease |
Q35203978 | Huntington's disease: a decade beyond gene discovery |
Q31105703 | Identification of benzothiazoles as potential polyglutamine aggregation inhibitors of Huntington's disease by using an automated filter retardation assay |
Q21144448 | Identification of potential therapeutic drugs for huntington's disease using Caenorhabditis elegans |
Q28554951 | In Vivo Modelling of ATP1A3 G316S-Induced Ataxia in C. elegans Using CRISPR/Cas9-Mediated Homologous Recombination Reveals Dominant Loss of Function Defects |
Q41497184 | In vitro aggregating β-lactamase-polyQ chimeras do not induce toxic effects in an in vivo Caenorhabditis elegans model. |
Q37261432 | Inclusion body myositis: a view from the Caenorhabditis elegans muscle |
Q36983190 | Inhibition of protein misfolding/aggregation using polyglutamine binding peptide QBP1 as a therapy for the polyglutamine diseases |
Q43782545 | Initial process of polyglutamine aggregate formation in vivo |
Q33938012 | Insulin signaling in the aging of healthy and proteotoxically stressed mechanosensory neurons |
Q35197814 | Invertebrate models of neurologic disease: insights into pathogenesis and therapy |
Q45300712 | Is there a cause-and-effect relationship between alpha-synuclein fibrillization and Parkinson's disease? |
Q46463399 | Isolation of gene sets affected specifically by polyglutamine expression: implication of the TOR signaling pathway in neurodegeneration |
Q36088674 | Kinetically competing huntingtin aggregation pathways control amyloid polymorphism and properties |
Q28554683 | Large-scale microfluidics providing high-resolution and high-throughput screening of Caenorhabditis elegans poly-glutamine aggregation model |
Q28077338 | Mechanisms of aging-related proteinopathies in Caenorhabditis elegans |
Q44155294 | Medical significance of Caenorhabditis elegans. |
Q45300220 | Mice transgenic for the human Huntington's disease mutation have reduced sensitivity to kainic acid toxicity |
Q37356257 | Mitochondrial structural and functional dynamics in Huntington's disease |
Q34408222 | Modeling Huntington's disease in cells, flies, and mice |
Q36339275 | Modeling age-related diseases in Drosophila: can this fly? |
Q37968206 | Modeling human neurodegenerative diseases in transgenic systems |
Q37293096 | Modeling molecular and cellular aspects of human disease using the nematode Caenorhabditis elegans |
Q79259216 | Modeling polyglutamine pathogenesis in C. elegans |
Q90359225 | Models and mechanisms of repeat expansion disorders: a worm's eye view |
Q50771820 | Modulation of prion-dependent polyglutamine aggregation and toxicity by chaperone proteins in the yeast model. |
Q37776087 | Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease |
Q34185510 | Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease |
Q37035950 | Morphological remodeling of C. elegans neurons during aging is modified by compromised protein homeostasis. |
Q85042543 | Muscleblind participates in RNA toxicity of expanded CAG and CUG repeats in Caenorhabditis elegans |
Q36065954 | Mutations in the pqe-1 gene enhance transgene expression in Caenorhabditis elegans |
Q37759354 | Neurodegenerative disorders: insights from the nematode Caenorhabditis elegans |
Q30502208 | Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathways |
Q36103669 | Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells |
Q33772869 | Neuroprotective effects and mechanism of cognitive-enhancing choline analogs JWB 1-84-1 and JAY 2-22-33 in neuronal culture and Caenorhabditis elegans |
Q34013543 | Neurotoxic effects of TDP-43 overexpression in C. elegans |
Q34514433 | No post-genetics era in human disease research |
Q37682750 | Non-apoptotic cell death in Caenorhabditis elegans |
Q30528027 | O-GlcNAc cycling mutants modulate proteotoxicity in Caenorhabditis elegans models of human neurodegenerative diseases |
Q37568290 | O-GlcNAc cycling shows neuroprotective potential in C. elegans models of neurodegenerative disease |
Q47068825 | Overexpression of dystrobrevin delays locomotion defects and muscle degeneration in a dystrophin-deficient Caenorhabditis elegans |
Q90439419 | Overview of Huntington's Disease Models: Neuropathological, Molecular, and Behavioral Differences |
Q36280482 | Pharmacological intervention in invertebrate aging. |
Q26860151 | Pluripotent stem cells models for Huntington's disease: prospects and challenges |
Q35746858 | Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans |
Q33703910 | Polyglutamine pathogenesis. |
Q79948907 | Polyglutamine proteins at the pathogenic threshold display neuron-specific aggregation in a pan-neuronal Caenorhabditis elegans model |
Q38248688 | Preclinical models: needed in translation? A Pro/Con debate. |
Q33571914 | Probing the metabolic aberrations underlying mutant huntingtin toxicity in yeast and assessing their degree of preservation in humans and mice |
Q33927179 | Proteases for cell suicide: functions and regulation of caspases |
Q36109387 | Protein Homeostasis in Models of Aging and Age-Related Conformational Disease |
Q45735441 | Protein Misfolding and Aggregation as a Therapeutic Target for Polyglutamine Diseases |
Q29614783 | Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging |
Q33650539 | Recent advances in understanding the pathogenesis of Huntington's disease |
Q37989861 | Role of the ubiquitin-proteasome system in nervous system function and disease: using C. elegans as a dissecting tool |
Q37688026 | Royal jelly promotes DAF-16-mediated proteostasis to tolerate β-amyloid toxicity in C. elegans model of Alzheimer's disease |
Q57182000 | Single copy/knock-in models of ALS SOD1 in C. elegans suggest loss and gain of function have different contributions to cholinergic and glutamatergic neurodegeneration |
Q34083380 | Studying human neurodegenerative diseases in flies and worms |
Q36005254 | Tales of cannibalism, suicide, and murder: Programmed cell death in C. elegans |
Q44604484 | Targeted nucleotide exchange in the CAG repeat region of the human HD gene |
Q35708355 | The Aggregation Inhibitor Peptide QBP1 as a Therapeutic Molecule for the Polyglutamine Neurodegenerative Diseases |
Q24616907 | The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis |
Q27301298 | The N17 domain mitigates nuclear toxicity in a novel zebrafish Huntington's disease model |
Q34219234 | The biochemistry of neuronal necrosis: rogue biology? |
Q34019143 | The early cellular pathology of Huntington's disease |
Q34308028 | The energetics of Huntington's disease |
Q28083445 | The role of insulin/IGF-1 signaling in the longevity of model invertebrates, C. elegans and D. melanogaster |
Q28069294 | The struggle by Caenorhabditis elegans to maintain proteostasis during aging and disease |
Q29619760 | The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans |
Q33891342 | Toward an understanding of polyglutamine neurodegeneration |
Q41821958 | Transforming Growth Factor-Beta Signaling in the Neural Stem Cell Niche: A Therapeutic Target for Huntington's Disease |
Q34890228 | Transgenic animal models of neurodegeneration based on human genetic studies. |
Q34340577 | Transgenic invertebrate models of age-associated neurodegenerative diseases |
Q89863572 | Unexpected cell type-dependent effects of autophagy on polyglutamine aggregation revealed by natural genetic variation in C. elegans |
Q38253642 | Use of Caenorhabditis elegans as a model to study Alzheimer's disease and other neurodegenerative diseases |
Q38089667 | Using C. elegans to decipher the cellular and molecular mechanisms underlying neurodevelopmental disorders |
Q34542345 | Using Caenorhabditis elegans as a model for aging and age-related diseases |
Q36687852 | Using Caenorhabditis elegans models of neurodegenerative disease to identify neuroprotective strategies |
Q50103667 | What Can We Learn About Human Disease from the Nematode C. elegans? |
Q37389590 | What have worm models told us about the mechanisms of neuronal dysfunction in human neurodegenerative diseases? |
Q35997893 | Xyloketal-derived small molecules show protective effect by decreasing mutant Huntingtin protein aggregates in Caenorhabditis elegans model of Huntington's disease |
Search more.