New light on polyglutamine neurodegenerative disorders: Interference with transcription

Trends in Molecular Medicine

Volumn 7, Issue 7, 2001, Pages 283-284

  Néri, Christian ^a  

^a FONDATION JEAN DAUSSET CEPH   (France)

Author keywords

[No Author keywords available]

Indexed keywords

CYCLIC AMP RESPONSIVE ELEMENT BINDING PROTEIN; GLUTAMINE; HUNTINGTIN; POLYGLUTAMINE; PROTEIN P300;

CADAVER; CELL DEATH; CELL NUCLEUS INCLUSION BODY; CYTOTOXICITY; DEGENERATIVE DISEASE; HUMAN; HUMAN TISSUE; HUNTINGTON CHOREA; INTERFEROMETER; NONHUMAN; PROTEIN CONFORMATION; PROTEIN LOCALIZATION; PROTEIN PROTEIN INTERACTION; RAT; SHORT SURVEY; TRANSCRIPTION REGULATION;

EID: 0034964020     PISSN: 14714914     EISSN: None     Source Type: Journal    
DOI: 10.1016/S1471-4914(01)02065-2     Document Type: Review

References (18)

1. Glutamine repeats and neurodegeneration
2. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
3. AYAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
4. Huntington aggregates may not predict neuronal death in Huntington's disease
5. Transgenic mice expressing mutated full-length HD cDNA: A paradigm for locomotor changes and selective neuronal loss in Huntington's disease
6. Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
7. Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice
8. Polyglutamine expansion downregulates specific neuronal genes before pathologic changes in SCA1
9. Amyloid formation by mutant huntingtin: Threshold, progressivity and recruitment of normal polyglutamine proteins
10. Aberrant interactions of transcriptional represser proteins with the Huntington's disease gene product, huntingtin
11. CREB-binding protein sequestration by expanded polyglutamine
12. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
13. Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription
14. The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: Neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis
15. Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity
16. Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells
17. Regulation of expanded polyglutamine protein aggregation and nuclear localization by the glucocorticoid receptor
18. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease