Huntington's disease: The challenge for cell biologists

Trends in Cell Biology

Volumn 10, Issue 12, 2000, Pages 531-536

  Tobin, Allan J ^a   Signer, Ethan R ^b  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b Hereditary Disease Foundation   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

POLYGLUTAMINE;

APOPTOSIS; DEGENERATIVE DISEASE; GENE OVEREXPRESSION; GENETIC TRANSCRIPTION; HUMAN; HUMAN CELL; HUNTINGTON CHOREA; MODEL; NEUROLOGIC DISEASE; NEUROPATHOLOGY; PRIORITY JOURNAL; PROTEIN DEGRADATION; REVIEW;

ANIMALS; APOPTOSIS; DRUG EVALUATION, PRECLINICAL; HUMANS; HUNTINGTON DISEASE; INCLUSION BODIES; NERVE TISSUE PROTEINS; NEURONS; NUCLEAR PROTEINS; PEPTIDES;

MUS MUSCULUS;

EID: 0034571171     PISSN: 09628924     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0962-8924(00)01853-5     Document Type: Review

References (34)

1. Trinucleotide repeats in neurogenetic disorders
2. Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?
3. Huntington aggregates may not predict neuronal death in Huntington's disease
4. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
5. Differential expression of normal and mutant Huntington's disease gene alleles
6. Mouse mutant embryos lacking huntingtin are rescued from lethality by wild-type extraembryonic tissues
7. Caspases and neurodegeneration: on the cutting edge of new therapeutic approaches
8. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
9. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
10. Nuclear targeting of mutant Huntingtin increases toxicity
11. Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition
12. A cellular model that recapitulates major pathogenic steps of Huntington's disease
13. Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology
14. Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei
15. Transglutaminase as the agent of neurodegenerative diseases due to polyglutamine expansion
16. Localization of rabbit huntingtin using a new panel of monoclonal antibodies
17. Mutant huntingtin forms in vivo complexes with distinct context-dependent conformations of the polyglutamine segment
18. Modeling protein homopolymeric repeats: possible polyglutamine structural motifs for Huntington's disease
19. Genetic neurodegenerative diseases: the human illness and transgenic models
20. Recent insights into the molecular pathogenesis of Huntington disease
21. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
22. Aggresomes: a cellular response to misfolded proteins
23. Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice
24. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
25. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice
26. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
27. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease
28. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of disease
29. Generation of neuronal intranuclear inclusions by polyglutamine-GFP: analysis of inclusion clearance and toxicity as a function of polyglutamine length
30. Expression of polyglutamine-expanded Huntingtin activates the SEK1-JNK pathway and induces apoptosis in a hippocampal neuronal cell line
31. Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease
32. Mitochondria in neurodegeneration: bioenergetic function in cell life and death
33. Decrease in striatal enkephalin mRNA in mouse models of Huntington's disease
34. Energetics in the pathogenesis of neurodegenerative diseases