Recent advances in Huntington's disease

Current Opinion in Neurology

Volumn 13, Issue 4, 2000, Pages 445-450

  Gutekunst, Claire Anne ^a   Norflus, Francine ^a   Hersch, Steven M ^a  

^a EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CASPASE INHIBITOR; HUNTINGTIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; APOPTOSIS; HUMAN; HUNTINGTON CHOREA; NEUROPATHOLOGY; NEUROPROTECTION; NONHUMAN; OXIDATIVE STRESS; REVIEW; TRANSGENIC MOUSE; TRINUCLEOTIDE REPEAT;

ANIMALS; APOPTOSIS; BRAIN; CELL DEATH; DISEASE MODELS, ANIMAL; HUMANS; HUNTINGTON DISEASE; MICE; MICE, KNOCKOUT; MICE, TRANSGENIC; NEUROLOGY; NEUROPROTECTIVE AGENTS;

EID: 0033851770     PISSN: 13507540     EISSN: None     Source Type: Journal    
DOI: 10.1097/00019052-200008000-00013     Document Type: Review

References (39)

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2. Neuropathology and pathophysiology of Huntington's disease. Movement disorders
3. Identification and localization of huntingtin in brain and lymphoblasts with anti-fusion protein antibodies
4. Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons
5. The cellular and subcellular localization of huntingtin-associated protein 1 (HAP1): Comparison with huntingtin in rat and human
6. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
7. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
8. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
9. Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
10. Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice
11. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
12. Huntingtin acts in nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
13. Mutant huntingtin expression in clonal striatal cells dissociation of inclusion formation and neuronal survival by caspase inhibition
14. Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
15. Huntington aggregates may not predict neuronal death in Huntington's disease
16. Genetic suppression of polyglutamine toxicity in Drosophila
17. Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins
18. Regulation of expanded polyglutamine protein aggregation and nuclear localization by the glucocorticoid receptor
19. Recent advances in understanding the pathogenesis of Huntington's disease
20. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
21. Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation
22. Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation
23. Detection of polyglutamine aggregation in mouse models
24. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease
25. Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes
26. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
27. Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA
28. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
29. A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice
30. Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease
31. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse
32. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny neurons in Hdh(Q92) and Hdh(Q111) knock-in mice
33. Intraneuronal aggregate formation and cell death after viral expression of expanded polyglutamine tracts in the adult rat brain
34. Oxidative stress in Huntington's disease
35. Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin
36. Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models
37. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
38. Caspase activation during apoptotic cell death induced by expanded polyglutamine in N2a cells
39. Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization