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Kaleidoscopic protein-protein interactions in the life and death of ataxin-1: new strategies against protein aggregation.

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.

SUBMITTER: de Chiara C

PROVIDER: S-EPMC3988977 | biostudies-literature | 2014 Apr

REPOSITORIES: biostudies-literature

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Kaleidoscopic protein-protein interactions in the life and death of ataxin-1: new strategies against protein aggregation.

de Chiara Cesira C Pastore Annalisa A

Trends in neurosciences 20140311 4

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 nonpatholo ...[more]

PMID: 24636457

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