Ataxin-3, DNA damage repair, and SCA3 cerebellar degeneration: on the path to parsimony?

  [...]ataxin-3 was shown to interact with the transcription factor FOXO4 as a transcriptional coactivator in the oxidative stress response [5]. [...]while some very basic functions of ataxin-3 have been established, these advances have yielded nonintersecting lines of investigation and have not pro...

Full description

Saved in:
Bibliographic Details
Published in:PLoS genetics 2015-01, Vol.11 (1), p.e1004937-e1004937
Main Authors: Ward, Jacqueline M, La Spada, Albert R
Format: Article
Language:English
Subjects:
Cerebellar ataxia
DNA damage
DNA Damage - genetics
DNA repair
DNA Repair Enzymes - genetics
Genetic aspects
Humans
Identification and classification
Kinases
Machado-Joseph Disease - genetics
Mutation
Nerve Tissue Proteins - genetics
Nuclear Proteins - genetics
Phosphotransferases (Alcohol Group Acceptor) - genetics
Repressor Proteins - genetics
Rodents
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:  [...]ataxin-3 was shown to interact with the transcription factor FOXO4 as a transcriptional coactivator in the oxidative stress response [5]. [...]while some very basic functions of ataxin-3 have been established, these advances have yielded nonintersecting lines of investigation and have not provided an underlying mechanism for SCA3 disease pathogenesis. A link between ataxin-3 and PNKP thus reinforces the importance of DNA repair in maintaining healthy cerebellar neurons and suggests targets for therapy development for SCA3 cerebellar degeneration, with possible application to other cerebellar degenerative diseases.
ISSN:1553-7404
1553-7390
1553-7404
DOI:10.1371/journal.pgen.1004937