Research Paper Volume 8, Issue 2 pp 382—393
Telomere shortening leads to earlier age of onset in ALS mice
- 1 Department of Neurology, Ulm University, 89081 Ulm, Germany
- 2 Department of Clinical & Biological Psychology, Ulm University, 89081 Ulm, Germany
- 3 Department of Internal Medicine III, Ulm University, 89081 Ulm, Germany
- 4 Leibniz Institute for Age Research, 07745 Jena, Germany
- 5 Department of Pathology, Ulm University, 89081 Ulm, Germany
Received: December 23, 2015 Accepted: January 27, 2016 Published: February 24, 2016
https://doi.org/10.18632/aging.100904How to Cite
Abstract
Telomere shortening has been linked to a variety of neurodegenerative diseases. Recent evidence suggests that reduced telomerase expression results in shorter telomeres in leukocytes from sporadic patients with amyotrophic lateral sclerosis (ALS) compared with healthy controls. Here, we have characterized telomere length in microglia, astroglia and neurons in human post mortem brain tissue from ALS patients and healthy controls. Moreover, we studied the consequences of telomerase deletion in a genetic mouse model for ALS. We found a trend towards longer telomeres in microglia in the brains of ALS patients compared to non-neurologic controls. Knockout of telomerase leading to telomere shortening accelerated the ALS phenotype in SOD1G93A–transgenic mice. Our results suggest that telomerase dysfunction might contribute to the age-related risk for ALS.