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Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats

Author

Listed:
  • M. F. Perutz

    (MRC Laboratory of Molecular Biology)

  • A. H. Windle

    (University of Cambridge)

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.

Suggested Citation

  • M. F. Perutz & A. H. Windle, 2001. "Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats," Nature, Nature, vol. 412(6843), pages 143-144, July.
    • Handle: RePEc:nat:nature:v:412:y:2001:i:6843:d:10.1038_35084141
    DOI: 10.1038/35084141
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    Cited by:

    1. Qi Wang & Joshua L Johnson & Nathalie YR Agar & Jeffrey N Agar, 2008. "Protein Aggregation and Protein Instability Govern Familial Amyotrophic Lateral Sclerosis Patient Survival," PLOS Biology, Public Library of Science, vol. 6(7), pages 1-19, July.
    2. Sreelakshmi Vasudevan & Subhadip Senapati & Maryanne Pendergast & Paul S.–H. Park, 2024. "Aggregation of rhodopsin mutants in mouse models of autosomal dominant retinitis pigmentosa," Nature Communications, Nature, vol. 15(1), pages 1-20, December.

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