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Article

The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation.

Proceedings of the National Academy of Sciences of the United States of America - 22 Mar 2005

Burnett Barrington G, Pittman Randall N

Abstract excerpt

The polyglutamine-containing neurodegenerative protein ataxin 3 (AT3) has deubiquitylating activity and binds ubiquitin chains with a preference for chains of four or more ubiquitins. Here we characterize the deubiquitylating activity of AT3 in vitro and show it trims/edits K48-linked ubiquitin chains. AT3 also edits polyubiquitylated (125)I-lysozyme and decreases its degradation by proteasomes. Cellular studies...

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Identifiers

PMID: 15767577
DOI: 10.1073/pnas.0407252102
PMCID: PMC555481

Genes

CFTR

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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