Huntington disease: New insights on the role of huntingtin cleavage

Journal of Neural Transmission, Supplement

Volumn , Issue 58, 2000, Pages 1-17

  Wellington, C L ^a   Leavitt, B R ^a   Hayden, M R ^a  

^a Centre for Molecular Medicine and Therapeutics   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

HUNTINGTIN;

AMINO TERMINAL SEQUENCE; CONFERENCE PAPER; HUMAN; HUNTINGTON CHOREA; NERVE DEGENERATION; PHENOTYPE; PRIORITY JOURNAL; TRANSGENIC ANIMAL; YEAST ARTIFICIAL CHROMOSOME;

EID: 0033708960     PISSN: 03036995     EISSN: None     Source Type: Book Series    
DOI: 10.1007/978-3-7091-6284-2_1     Document Type: Conference Paper

References (30)

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3. Truncated N-terminal fragments of huntingtin with expanded glutamine repeats from nuclear and cytoplasmic aggregates in vitro
4. Formation of neuronal intranuclear inclusions (NII) underlies the neurological dysfunction in mice transgenic for the HD mutation
5. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
6. Trinucleotide repeat length instability and age of onset in Huntington disease
7. Kennedy's disease: Caspase cleavege of the androgen receptor is a crucial event in cytotoxicity
8. Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract
9. The influence of huntingtin protein size on nuclear localization and cellular toxicity
10. Human huntingtin derived from YAC transgenes compensates for loss of murine huntingtin by rescue of the embryonic lethal phenotype
11. A YAC mouse model for Huntington's disease with full-length mutant huntingtin cytoplasmic toxicity and selective striatal neurodegeneration
12. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
13. Ataxin-1 nuclear localization and aggregation: Role n polyglutamine-induced disease in SCA1 transgenic mice
14. Recent insights into the pathogenesis of Huntington Disease
15. A cellular model that recapitulates major pathogenetic steps of Huntingtons Disease
16. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
17. Length of the protein and polyglutamine tract influence localization and frequency of intracellular aggregates of huntingtin
18. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
19. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3
20. Behavioral abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA
21. The neuropathology of CAG repeat diseases: Review and update of genetic and molecular features
22. Caspase-8 is required for cell death induced by expanded polyglutamine repeats
23. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
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26. Of molecular interactions mice and mechanisms: New insights into Huntington's disease
27. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
28. Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells