Journal
HUMAN MOLECULAR GENETICS
Volume 21, Issue 1, Pages 57-65Publisher
OXFORD UNIV PRESS
DOI: 10.1093/hmg/ddr437
Keywords
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Funding
- China Scholarship Council
- National Ataxia Foundation
- National Natural Science Foundation of China [30430260, 30225024]
- NIH [R01 NS051630, R21 NS067461]
- Beckman Young Investigator Award
- Basil O'Connor Scholar Research Award
- NATIONAL INSTITUTE OF MENTAL HEALTH [R01MH076090] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS051630, R21NS067461] Funding Source: NIH RePORTER
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Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder associated with fragile X premutation carriers. Previous studies have shown that fragile X rCGG repeats are sufficient to cause neurodegeneration and that the rCGG-repeat-binding proteins Pur a and heterogeneous nuclear ribonucleoprotein (hnRNP) A2/B1 could modulate rCGG-mediated neuronal toxicity. Mobile genetic elements or their remnants populate the genomes, and the activities of these elements are tightly controlled for the fitness of host genomes in different organisms. Here we provide both biochemical and genetic evidence to show that the activation of a specific retrotransposon, gypsy, can modulate rCGG-mediated neurodegeneration in an FXTAS Drosophila model. We find that one of the rCGG-repeat-binding proteins, hnRNP A2/B1, is involved in this process via interaction with heterochromatin protein 1. Knockdown of gypsy RNA by RNAi could suppress the neuronal toxicity caused by rCGG repeats. These data together point to a surprisingly active role for retrotransposition in neurodegeneration.
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