Mol Neurodegener. 2015 Jun 10;10(1):22. doi: 10.1186/s13024-015-0019-6.
Anjomani Virmouni S1,2, Al-Mahdawi S3,4, Sandi C5,6, Yasaei H7, Giunti P8, Slijepcevic P9, Pook MA10,11.
RESULTS:
Here we aimed to investigate both telomere structure and function in FRDA cells. Our results confirmed telomere shortening in FRDA patient leukocytes and identified similar telomere shortening in FRDA patient autopsy cerebellar tissues. However, FRDA fibroblasts showed significantly longer telomeres at early passage, occurring in the absence of telomerase activity, but with activation of an alternative lengthening of telomeres (ALT)-like mechanism.
Here we aimed to investigate both telomere structure and function in FRDA cells. Our results confirmed telomere shortening in FRDA patient leukocytes and identified similar telomere shortening in FRDA patient autopsy cerebellar tissues. However, FRDA fibroblasts showed significantly longer telomeres at early passage, occurring in the absence of telomerase activity, but with activation of an alternative lengthening of telomeres (ALT)-like mechanism.
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