Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats
- 1 July 2001
- journal article
- Published by Springer Nature in Nature
- Vol. 412 (6843) , 143-144
- https://doi.org/10.1038/35084141
Abstract
Neurodegenerative diseases resulting from expanded repeat sequences of glutamine residues are associated with the formation of protein aggregates in the cell nuclei of the affected neurons, but whether these are pathogenic is controversial. Recent observations indicate that the ages of onset of these diseases are exponential functions of the repeat lengths and that the probability of neural death is constant with time. The only process known to us that could give rise to such behaviour is nucleation of the aggregates.Keywords
This publication has 14 references indexed in Scilit:
- Interference by Huntingtin and Atrophin-1 with CBP-Mediated Transcription Leading to Cellular ToxicityScience, 2001
- Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's diseaseProceedings of the National Academy of Sciences, 2000
- A one-hit model of cell death in inherited neuronal degenerationsNature, 2000
- Reversal of Neuropathology and Motor Dysfunction in a Conditional Model of Huntington's DiseaseCell, 2000
- A molecular investigation of true dominance in Huntington's diseaseJournal of Medical Genetics, 1999
- Glutamine repeats and neurodegenerative diseases: molecular aspectsTrends in Biochemical Sciences, 1999
- Huntingtin Acts in the Nucleus to Induce Apoptosis but Death Does Not Correlate with the Formation of Intranuclear InclusionsCell, 1998
- Amyloid Formation by Mutant Huntingtin: Threshold, Progressivity and Recruitment of Normal Polyglutamine ProteinsSomatic Cell and Molecular Genetics, 1998
- Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in BrainScience, 1997
- Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases.Proceedings of the National Academy of Sciences, 1994