Therapeutic opportunities in polyglutamine disease

Nature Medicine

Volumn 7, Issue 4, 2001, Pages 419-423

  Hughes, Robert E ^a   Olson, James M ^b  

^a UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

^b SEATTLE CHILDREN S RESEARCH INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

POLYGLUTAMINE;

DEGENERATIVE DISEASE; DRUG TARGETING; FAMILIAL DISEASE; HUNTINGTON CHOREA; NEUROPATHOLOGY; PRIORITY JOURNAL; PROGNOSIS; PROTEIN EXPRESSION; PROTEIN FOLDING; REVIEW;

ANIMALS; APOPTOSIS; CASPASES; DRUG DESIGN; HUMANS; HUNTINGTON DISEASE; MOLECULAR CHAPERONES; NERVE DEGENERATION; NEURODEGENERATIVE DISEASES; PEPTIDES; PROTEIN FOLDING; TRANSCRIPTION, GENETIC; TRINUCLEOTIDE REPEATS;

EID: 0035047651     PISSN: 10788956     EISSN: None     Source Type: Journal    
DOI: 10.1038/86486     Document Type: Review

References (56)

1. Glutamine repeats and neurodegeneration
2. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
3. Evidence for proteasome involvement in polyglutamine disease: Localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro
4. Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone
5. Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease
6. Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract
7. Hsp70 and Hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils
8. Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans
9. Identification of genes that modify ataxin-1-induced neurodegeneration
10. Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70
11. Genetic suppression of polyglutamine toxicity in Drosophila
12. Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease
13. Ubiquitin, cellular inclusions and their role in neurodegeneration
14. Protein fate in neurodegenerative proteinopathies: Polyglutamine diseases join the (mis)fold
15. Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice
16. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
17. A huntingtin-associated protein enriched in brain with implications for pathology
18. HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane- associated huntingtin in the brain
19. SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates
20. Huntingtin interacts with a family of WW domain proteins
21. Trinucleotide repeats are clustered in regulatory genes in Saccharomyces cerevisiae
22. Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation
23. Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin
24. Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells
25. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
26. CREB-binding protein sequestration by expanded polyglutamine
27. Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription
28. Transcriptional dysregulation in Huntington's disease
29. Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1
30. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease
31. Lesions of striatal neurones with kainic acid provides a model for Huntington's chorea
32. Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic acid and kainic acids
33. Replicating Huntington's disease phenotype in experimental animals
34. Oxidative stress, glutamate and neurodegenerative disorders
35. Inhibition of succinate dehydrogenase by malonic acid produces an "excitotoxic" lesion in rat striatum
36. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease
37. Antiglutamate therapies in Huntington's disease
38. Influence of lamotrigine on progression of early Huntington disease: A randomized clinical trial
39. Cognitive, behavioral, and motor effects of the NMDA antagonist ketamine in Huntington's disease
40. A controlled clinical trial of baclofen as protective therapy in early Huntington's disease
41. The inflammatory response system of brain: Implications for therapy of Alzheimer and other neurodegenerative diseases
42. Increased complement biosynthesis by microglia and complement activation on neurons in Huntington's disease
43. Transplanted fetal striatum in Huntington's disease: Phenotypic development and lack of pathology
44. Ibuprofen suppresses plaque pathology and inflammation in a mouse model for Alzheimer's disease
45. Inflammation and Alzheimer's disease
46. Caspases and neurodegeneration: On the cutting edge of new therapeutic approaches
47. In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes
48. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
49. The influence of huntingtin protein size on nuclear localization and cellular toxicity
50. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
51. Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells
52. Caspase-8 is required for cell death induced by expanded polyglutamine repeats
53. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
54. Bimoclomol: A nontoxic, hydroxylamine derivative with stress protein-inducing activity and cytoprotective effects
55. Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: Implications for Huntington's disease therapy
56. Expanded polyglutamine peptides alone are intrinsically cytotoxic and cause neurodegeneration in Drosophila