When more is less: pathogenesis of glutamine repeat neurodegenerative diseases.
about
Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicityTowards a unifying, systems biology understanding of large-scale cellular death and destruction caused by poorly liganded iron: Parkinson's, Huntington's, Alzheimer's, prions, bactericides, chemical toxicology and others as examplesAtrophin-1, the dentato-rubral and pallido-luysian atrophy gene product, interacts with ETO/MTG8 in the nuclear matrix and represses transcriptionCloning of the SCA7 gene reveals a highly unstable CAG repeat expansionHuntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzymeA human HAP1 homologue. Cloning, expression, and interaction with huntingtinCloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeatsHIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brainAtaxin-1 with an expanded glutamine tract alters nuclear matrix-associated structuresHuntingtin-associated protein (HAP1): discrete neuronal localizations in the brain resemble those of neuronal nitric oxide synthaseEvidence for a major retinitis pigmentosa locus on 19q13.4 (RP11) and association with a unique bimodal expressivity phenotypeThe localization and interactions of huntingtinTransgenic models of Huntington's diseaseCaspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tractHAP1-huntingtin interactions do not contribute to the molecular pathology in Huntington's disease transgenic miceNeuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathwaysPredicting Disease Onset from Mutation Status Using Proband and Relative Data with Applications to Huntington's Disease.Molecular and cellular genetics of fragile X syndrome.Exclusion of CAG repeat expansion as the cause of disease in autosomal dominant retinitis pigmentosa families.Analysis of spinocerebellar ataxia types 1, 2, 3, and 6, dentatorubral-pallidoluysian atrophy, and Friedreich's ataxia genes in spinocerebellar ataxia patients in the UK.Polyglutamine pathogenesis.CAG-polyglutamine-repeat mutations: independence from gene context.Widespread occurrence of intranuclear atrophin-1 accumulation in the central nervous system neurons of patients with dentatorubral-pallidoluysian atrophy.Practical approaches to determining disease-susceptible loci in multigenic autoimmune models.Pathology of CAG repeat diseases.The selective vulnerability of nerve cells in Huntington's disease.The mammalian degenerin MDEG, an amiloride-sensitive cation channel activated by mutations causing neurodegeneration in Caenorhabditis elegans.Polyglutamine fibrillogenesis: the pathway unfoldsWild-type but not mutant huntingtin modulates the transcriptional activity of liver X receptorsStriatal pre-enkephalin overexpression improves Huntington's disease symptoms in the R6/2 mouse model of Huntington's diseaseGenotype-phenotype correlation in CAG-repeat diseases.Spinocerebellar ataxia type 7 associated with pigmentary retinal dystrophy.Toward understanding Machado-Joseph diseasePhenotypic 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.Dithiol-based compounds maintain expression of antioxidant protein peroxiredoxin 1 that counteracts toxicity of mutant huntingtin.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.RNAi: a potential therapy for the dominantly inherited nucleotide repeat diseases.Spinocerebellar ataxia type 6: genotype and phenotype in German kindredsPolyglutamine disease: recent advances in the neuropathology of dentatorubral-pallidoluysian atrophy.Mechanisms mediating spinal and bulbar muscular atrophy: investigations into polyglutamine-expanded androgen receptor function and dysfunction.
P2860
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P2860
When more is less: pathogenesis of glutamine repeat neurodegenerative diseases.
description
1995 nî lūn-bûn
@nan
1995年の論文
@ja
1995年論文
@yue
1995年論文
@zh-hant
1995年論文
@zh-hk
1995年論文
@zh-mo
1995年論文
@zh-tw
1995年论文
@wuu
1995年论文
@zh
1995年论文
@zh-cn
name
When more is less: pathogenesis of glutamine repeat neurodegenerative diseases.
@en
type
label
When more is less: pathogenesis of glutamine repeat neurodegenerative diseases.
@en
prefLabel
When more is less: pathogenesis of glutamine repeat neurodegenerative diseases.
@en
P1433
P1476
When more is less: pathogenesis of glutamine repeat neurodegenerative diseases.
@en
P2093
P304
P356
10.1016/0896-6273(95)90138-8
P407
P577
1995-09-01T00:00:00Z