Abstract |
Understanding how proteins protect themselves from aberrant aggregation is of primary interest for understanding basic biology, protein biochemistry, and human disease. We discuss the paradigmatic example of ataxin-1 (Atx1), the protein responsible for neurodegenerative spinocerebellar ataxia type 1 ( SCA1). This disease is part of the increasing family of pathologies caused by protein aggregation and misfolding. We discuss the importance of protein- protein interactions not only in the nonpathological function of Atx1 but also in protecting the protein from aggregation and misfolding. The lessons learned from Atx1 may lead to a more general understanding of the cell's protective strategies against aggregation. The obtained knowledge may suggest a new perspective for designing specific therapeutic strategies for the cure of misfolding diseases.
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Authors | Cesira de Chiara, Annalisa Pastore |
Journal | Trends in neurosciences
(Trends Neurosci)
Vol. 37
Issue 4
Pg. 211-8
(Apr 2014)
ISSN: 1878-108X [Electronic] England |
PMID | 24636457
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
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Copyright | Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved. |
Chemical References |
- ATXN1 protein, human
- Ataxin-1
- Ataxins
- Nerve Tissue Proteins
- Nuclear Proteins
- Peptides
- Protein Aggregates
- polyglutamine
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Topics |
- Animals
- Ataxin-1
- Ataxins
- DNA Repeat Expansion
- Drug Design
- Heredodegenerative Disorders, Nervous System
(drug therapy, genetics, metabolism)
- Humans
- Nerve Tissue Proteins
(chemistry, genetics, metabolism)
- Nuclear Proteins
(chemistry, genetics, metabolism)
- Peptides
(genetics, metabolism)
- Protein Aggregates
- Protein Aggregation, Pathological
- Protein Folding
- Protein Multimerization
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