Polyglutamines, nuclear inclusions and neurodegeneration. - Wikidata - wikimedia - TriplyDB

Polyglutamines, nuclear inclusions and neurodegeneration.

Polyglutamines, nuclear inclusions and neurodegeneration.

http://www.wikidata.org/entity/Q45295249

about

Pathogenesis of inclusion bodies in (CAG)n/Qn-expansion diseases with special reference to the role of tissue transglutaminase and to selective vulnerability.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.Molecular clearance of ataxin-3 is regulated by a mammalian E4 Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation.Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei.UBE4B: a promising regulatory molecule in neuronal death and survival.Genetic variants in diseases of the extrapyramidal system.PGC-1α at the intersection of bioenergetics regulation and neuron function: from Huntington's disease to Parkinson's disease and beyond.CAG repeats mimic CUG repeats in the misregulation of alternative splicing.Intracellular processing and toxicity of the truncated androgen receptor: nuclear congophilia-associated cell death.Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro.Polyglutamine-induced ion channels: a possible mechanism for the neurotoxicity of Huntington and other CAG repeat diseases.SUMO-Targeted Ubiquitin Ligases (STUbLs) Reduce the Toxicity and Abnormal Transcriptional Activity Associated With a Mutant, Aggregation-Prone Fragment of Huntingtin Variation on a trinucleotide theme

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Polyglutamines, nuclear inclusions and neurodegeneration.

http://www.wikidata.org/entity/Q45295249

description

1997 nî lūn-bûn

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1997年の論文

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1997年学术文章

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1997年学术文章

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1997年学术文章

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1997年学术文章

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1997年学术文章

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1997年学术文章

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1997年學術文章

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1997年學術文章

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name

Polyglutamines, nuclear inclusions and neurodegeneration.

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Polyglutamines, nuclear inclusions and neurodegeneration.

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type

label

Polyglutamines, nuclear inclusions and neurodegeneration.

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Polyglutamines, nuclear inclusions and neurodegeneration.

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prefLabel

Polyglutamines, nuclear inclusions and neurodegeneration.

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Polyglutamines, nuclear inclusions and neurodegeneration.

@nl

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Nature Medicine

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Polyglutamines, nuclear inclusions and neurodegeneration.

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A Lunkes

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J L Mandel

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P2860

HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

Trinucleotide repeats in neurogenetic disorders.

Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract.

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias.

Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo.

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

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1201-1202

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scholarly article

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10.1038/NM1197-1201

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11

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9359692

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