Inhibition of polyglutamine aggregation in R6/2 HD brain slices - Complex dose-response profiles

Neurobiology of Disease

Volumn 8, Issue 6, 2001, Pages 1017-1026

  Smith, Donna L ^a   Portier, Ruben ^b   Woodman, Ben ^a   Hockly, Emma ^a   Mahal, Amarbirpal ^a   Klunk, William E ^c   Li, Xiao Jiang ^d   Wanker, Erich ^e   Murray, Karl D ^f   Bates, Gillian P ^a  

^a KING'S COLLEGE LONDON   (United Kingdom)

^b RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^c UNIVERSITY OF PITTSBURGH   (United States)

^d EMORY UNIVERSITY   (United States)

^e MAX PLANCK INSTITUTE FOR MOLECULAR GENETICS   (Germany)

^f UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CONGO RED; CREATINE; POLYGLUTAMINE;

ANIMAL TISSUE; ARTICLE; BRAIN SLICE; CONCENTRATION RESPONSE; CONTROLLED STUDY; HUNTINGTON CHOREA; IMMUNOHISTOCHEMISTRY; MOUSE; NEUROPATHOLOGY; NONHUMAN; NUCLEOTIDE REPEAT; PHENOTYPE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN FOLDING;

EID: 0035668573     PISSN: 09699961     EISSN: None     Source Type: Journal    
DOI: 10.1006/nbdi.2001.0438     Document Type: Article

References (44)

1. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: Correlation between the density of inclusions and IT15 CAG triplet repeat length
2. Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation
3. Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease
4. Subtype-specific enhancement of NMDA receptor currents by mutant huntingtin
5. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
6. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
7. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
8. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease
9. Amyloid deposits and amyloidosis. The beta-fibrilloses
10. Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
11. Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: Implications for Huntington's disease therapy
12. Congo red prolongs the incubation period in scrapie-infected hamsters
13. Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: Their role in suppression of aggregation and cellular toxicity
14. Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy
15. Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells
16. Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
17. Chrysamine-G, a lipophilic analogue of Congo red, inhibits A beta-induced toxicity in PC12 cells
18. Development of small molecule probes for the β-amyloid protein of Alzheimer's disease
19. Bis azo dye liquid crystalline micelles as possible drug carriers in immuno-targeting technique
20. Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease
21. Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice
22. Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats
23. Mad cows meet psi-chotic yeast: The expansion of the prion hypothesis
24. Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation
25. Beta-amyloid neurotoxicity requires fibril formation and is inhibited by congo red
26. Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation
27. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
28. CREB-binding protein sequestration by expanded polyglutamine
29. Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils
30. Huntington's disease
31. Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity
32. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
33. Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation
34. Glutamine repeats as polar zippers: Their possible role in inherited neurodegenerative diseases
35. Screening Congo red and its analogues for their ability to prevent the formation of PrP-res in scrapie-infected cells
36. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
37. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
38. Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: Implications for Huntington's disease pathology
39. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
40. A simple method for organotypic cultures of nervous tissue
41. Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse
42. Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation
43. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease