Reducing mitochondrial ROS improves disease-related pathology in a mouse model of ataxia-telangiectasia
-
Altmetric Citations
D'Souza, Anthony D; Parish, Ian; Krause, Diane S.; Kaech, Susan; Shadel, Gerald S
Description
The disease ataxia-telangiectasia (A-T) has no cure and few treatment options. It is caused by mutations in the ATM kinase, which functions in the DNA-damage response and redox sensing. In addition to severe cerebellar degeneration, A-T pathology includes cancer predisposition, sterility, immune system dysfunction, and bone marrow abnormalities. These latter phenotypes are recapitulated in the ATM null (ATM-/-) mouse model of the disease. Since oxidative stress and mitochondrial dysfunction are...[Show more]
Collections | ANU Research Publications |
---|---|
Date published: | 2013 |
Type: | Journal article |
URI: | http://hdl.handle.net/1885/77434 |
Source: | Molecular therapy : the journal of the American Society of Gene Therapy |
DOI: | 10.1038/mt.2012.203 |
Download
File | Description | Size | Format | Image |
---|---|---|---|---|
01_D'Souza_Reducing_mitochondrial_ROS_2013.pdf | 471.36 kB | Adobe PDF | Request a copy |
Items in Open Research are protected by copyright, with all rights reserved, unless otherwise indicated.
Updated: 19 May 2020/ Responsible Officer: University Librarian/ Page Contact: Library Systems & Web Coordinator