Journal home
Advance online publication
Current issue
Archive
Press releases
Supplements
Focuses
Guide to authors
Online submissionOnline submission
For referees
Free online issue
Contact the journal
Subscribe
Advertising
work@npg
Reprints and permissions
About this site
For librarians
 
NPG Resources
Nature
Nature Reviews
Nature Immunology
Nature Cell Biology
Nature Genetics
news@nature.com
Nature Conferences
Dissect Medicine
NPG Subject areas
Biotechnology
Cancer
Chemistry
Clinical Medicine
Dentistry
Development
Drug Discovery
Earth Sciences
Evolution & Ecology
Genetics
Immunology
Materials Science
Medical Research
Microbiology
Molecular Cell Biology
Neuroscience
Pharmacology
Physics
Browse all publications
News and Views
Nature Medicine  3, 1201 - 1202 (1997)
doi:10.1038/nm1197-1201

Polyglutamines, nuclear inclusions and neurodegeneration

Astrid Lunkes & Jean-Louis Mandel

Institut de Genetique etBiologie Moleculaire, Et Cellulaire CNRS/INSERM/Université Louis Pasteur, BP163,67404 Hlkirch Cedex, CU, Strasbourg France

Amyloid-like aggregates of expanded polyglutamines are present in nuclei from affected neurons in Huntington and related neurodegenerative diseases.

REFERENCES
  1. Paulson H.L. & Fishbeck, K.H. Trinucleotide repeats in neurogenetic disorders. Ann. Rev. Neurosci. 19, 79−107 (1997). | Article | ISI |
  2. Zoghbi, H.Y. CAG repeats in SCA6: anticipating new clues. Neurology in the press.
  3. Davies, S.W. et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537−548 (1997). | Article | PubMed  | ISI | ChemPort |
  4. Mangiarini, L. et al. Exon l of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87, 493−506 (1996). | Article | PubMed  | ISI | ChemPort |
  5. Skinner, P.J. et al. SCA1 pathogenesis involves alterations in nuclear matrix-associated structures. Nature in the press.
  6. Difiglia, M. et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990−1993 (1997). | Article | PubMed  | ISI | ChemPort |
  7. Paulson, H.L. et al. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19, 333−344 (1997). | Article | PubMed  | ISI | ChemPort |
  8. Clark, H.B. et al. Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviours followed by a progressive cerebellar dysfunction and histologies! alterations. J. Neurosci. in the press.
  9. Matilla, A. et al. The cerebellar leucine-rich acidic nuclear protein (LANP) interacts with ataxin-l. Nature in the press.
  10. Kalchman, M.A. et al. HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain. Nature Genet 16, 44−53 (1997). | Article | PubMed  | ISI | ChemPort |
  11. Jarrett, J.T. & Lansbury, P.T. Seeding "one-dimensional crystallization" of amyloid: A pathogenic mechanism in Alzheimer's disease and scrapie. Cell 73, 1055−1058 (1993). | PubMed  | ISI | ChemPort |
  12. Goldberg, Y.P. et al. Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nature Genet. 13, 442−449 (1996). | Article | PubMed  | ISI | ChemPort |
  13. Scherzinger, E. et al. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90, 549−558 (1997). | Article | PubMed  | ISI | ChemPort |
  14. Stott, K., Blackburn, J.M., Butler, P.J. & Perutz, M. Incorporation of glutamine repeats make proteins oligomerize: Implications for neurodegenerative diseases. Proc. Notl. Acad. Sci. U.S.A. 92, 6509−6513 (1995). | ChemPort |
  15. Trottier, Y. et al. Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias. Nature 378, 403−406 (1995). | Article | PubMed  | ISI | ChemPort |
 Top
 Top
References
Previous | Next
Table of contents
Download PDFDownload PDF
Send to a friendSend to a friend
Save this linkSave this link

Open Innovation Challenges

naturejobs

References
Export citation
Export references
natureproducts

Search buyers guide:

 
ADVERTISEMENT
 
Nature Medicine
ISSN: 1078-8956
EISSN: 1546-170X
Journal home | Advance online publication | Current issue | Archive | Press releases | Supplements | Focuses | For authors | Online submission | For referees | Free online issue | About the journal | Contact the journal | Subscribe | Advertising | work@npg | Reprints and permissions | About this site | For librarians
Nature Publishing Group, publisher of Nature, and other science journals and reference works©1997 Nature Publishing Group | Privacy policy