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Chaperones in Polyglutamine Aggregation: Beyond the Q-Stretch.
E F E Kuiper1, Eduardo P de Mattos2, Laura B Jardim3
1Department of Cell Biology, University Medical Center Groningen, University of Groningen Groningen, Netherlands.
Frontiers in Neuroscience
|April 8, 2017
Summary
Expanded polyglutamine (polyQ) stretches cause inherited neurodegenerative diseases. Factors like flanking sequences and molecular chaperones modulate polyQ protein aggregation, explaining disease variability.
Area of Science:
- Neuroscience
- Genetics
- Molecular Biology
Background:
- Expanded polyglutamine (polyQ) stretches in proteins cause inherited neurodegenerative disorders.
- PolyQ expansion size correlates with disease onset and protein aggregation.
Purpose of the Study:
- To review factors influencing polyQ aggregation and disease variability.
- To explore the role of molecular chaperones in polyQ protein handling.
Main Methods:
- Literature review of factors affecting polyQ aggregation.
- Analysis of polyQ diseases, expansion thresholds, and age at onset.
- Focus on molecular chaperones and protein quality control.
Main Results:
- PolyQ expansion length is a primary driver of disease onset.
- Flanking sequences, protein interactions, cleavage, and post-translational modifications modulate aggregation.
- Molecular chaperones impact the handling of polyQ proteins.
Conclusions:
- Variability in polyQ disease onset is influenced by multiple factors beyond expansion size.
- Understanding these factors provides molecular insights into disease pathogenesis.
- Molecular chaperones play a critical role in mitigating polyQ-induced neurodegeneration.