Involvement of polyglutamine endolysis followed by pyroglutamate formation in the pathogenesis of triplet repeat/polyglutamine-expansion diseases

Medical Hypotheses

Volumn 54, Issue 3, 2000, Pages 427-429

  Saido, T C ^a  

^a RIKEN BRAIN SCIENCE INSTITUTE   (Japan)

Author keywords

[No Author keywords available]

Indexed keywords

POLYGLUTAMINE; PYROGLUTAMIC ACID;

DEGENERATIVE DISEASE; GENE MUTATION; HUMAN; HYDROPHOBICITY; PRIORITY JOURNAL; SHORT SURVEY; TRINUCLEOTIDE REPEAT;

EID: 0034127807     PISSN: 03069877     EISSN: None     Source Type: Journal    
DOI: 10.1054/mehy.1999.0866     Document Type: Article

References (14)

1. Ashley C. T. Jr, Warren S. T. Trinucleotide repeat expansion and human disease. Ann Rev Genet. 29:1995;703-728.
2. Creighton T. E. Proteins, Structures and Molecular Properties, 2nd edn. 1993;W. H. Freeman, New York. p. 6-20.
3. Koger J. B., Humm J., Kizer J. S. Assay of glutaminylpeptide cyclase. Methods Enzymol. 168:1989;358-365.
4. McDonald J. K., Barret A. J. Mammalian Proteases: A Glossary and Bibliography Vol. 1: Endopeptidases. 1980;Academic Press, London.
5. Scherzinger E., Lurz R., Turmaine M. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell. 90:1997;549-558.
6. Davies S. W., Turmaine M., Cozens B. A. Formation of intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell. 90:1997;537-548.
7. Paulson H. L., Perez M. K., Trottier Y. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron. 19:1997;333-344.
8. Jarret J. T., Lansbury P. T. Jr Seeding 'one-dimensional crystallization' of amyloid: a pathological mechanism in Alzheimer's disease and scrapie? Cell. 73:1993;1055-1058.
9. Klement I. A., Skinner P. J., Kaytor M. D. Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell. 95:1998;41-53.
10. Saudou F., Finkbeiner S., Devys D. Hutington acts in the nucleus to induce apoptosis but death does not correlate with the formation of intracellular inclusions. Cell. 95:1998;55-66.
11. Saido T. C., Iwatsubo T., Mann D. M. Dominant and differential deposition of distinct β-amyloid peptide species, AβN3(pE), in senile plaques. Neuron. 14:1995;457-466.
12. Saido T. C., Yamao-Harigaya W., Iwatsubo T. Amino- and carboxyl-terminal heterogeneity of β-amyloid peptides deposited in human brain. Neurosci Lett. 215:1996;173-176.
13. Kuo Y. M., Emmerling M. R., Woods A. S. Biochem Biophys Res Commun. 237:1997;188-191.
14. Ikeda H., Yamaguchi M., Sugai S. Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo. Nature Genet. 13:1996;196-202.