Tissue-specific proteolysis of huntingtin (htt) in human brain: Evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum

Journal of Neuroscience

Volumn 21, Issue 6, 2001, Pages 1830-1837

  Mende Mueller, Liane M ^a,d   Toneff, Thomas ^a,b   Hwang, Shin Rong ^a,b   Chesselet, Marie Francoise ^c   Hook, Vivian Y H ^a,b  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b BUCK INSTITUTE FOR RESEARCH ON AGING   (United States)

^c UNIVERSITY OF CALIFORNIA   (United States)

^d Biotechnology Facility   (United States)

Author keywords

Brain; Huntingtin; Huntington's disease; Neurodegenerative disease; Proteolytic fragments; Ubiquitin

Indexed keywords

HUNTINGTIN; REPETITIVE DNA; UBIQUITIN;

ADULT; AGED; AMINO TERMINAL SEQUENCE; ARTICLE; BRAIN CORTEX; CARBOXY TERMINAL SEQUENCE; CONTROLLED STUDY; CORPUS STRIATUM; GENE CONTROL; HUMAN; HUMAN TISSUE; HUNTINGTON CHOREA; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN DOMAIN; PROTEIN MODIFICATION; PUTAMEN; TISSUE SPECIFICITY;

EID: 0035869544     PISSN: 02706474     EISSN: None     Source Type: Journal    
DOI: 10.1523/jneurosci.21-06-01830.2001     Document Type: Article

References (24)

1. Transgenic models of Huntington's disease
2. Expression of normal and mutant huntingtin in the developing brain
3. Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation
4. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic of the HD mutation
5. Morphometric demonstration of atrophic changes in the cerebral cortex, white matter and neostriatum in Huntington's disease
6. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
7. Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract
8. Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
9. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
10. Immunochemical characterization of carboxypeptidase B-like peptide hormone processing enzyme
11. Evidence for functional localization of the proenkephalinprocessing enzyme, prohormone thiol protease, to secretory vesicles of chromaffin cells
12. The Kunitz protease inhibitor form of the amyloid precursor protein (KPI/APP) inhibits the proneuropeptide processing enzyme prohormone thiol protease (PTP)
13. 1-antichymotrypsin
14. 1-antichymotrypsin (ACT) protease inhibitor in Alzheimer's disease brains: Comparison of ACT in hippocampus and liver
15. Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme
16. Mutant huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition
17. Huntington's disease gene (IT15) is widely expressed in human and rat tissues
18. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
19. Recent advances on the pathogenesis of Huntington's disease
20. Homozygotes and heterozygotes for ciliary neurotrophic factor null alleles do not show earlier onset of Huntington's disease
21. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
22. Huntington disease
23. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
24. Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells