Replication-independent instability of Friedreich's ataxia GAA repeats during chronological aging.

ABSTRACT: Nearly 50 hereditary diseases result from the inheritance of abnormally long repetitive DNA microsatellites. While it was originally believed that the size of inherited repeats is the key factor in disease development, it has become clear that somatic instability of these repeats throughout an individual's lifetime strongly contributes to disease onset and progression. Importantly, somatic instability is commonly observed in terminally differentiated, postmitotic cells, such as neurons. To unravel the mechanisms of repeat instability in nondividing cells, we created an experimental system to analyze the mutability of Friedreich's ataxia (GAA)_n repeats during chronological aging of quiescent Saccharomyces cerevisiae Unexpectedly, we found that the predominant repeat-mediat

SUBMITTER: Neil AJ 

PROVIDER: S-EPMC7865128 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature