Article

Neuropathology of RAN translation proteins in Fragile X-associated Tremor/Ataxia Syndrome

2019-06-15

Abstract excerpt

CGG repeat expansions in FMR1 cause the neurodegenerative disorder Fragile X-associated Tremor/Ataxia Syndrome (FXTAS). Ubiquitinated neuronal intranuclear inclusions (NIIs) are the neuropathological hallmark of FXTAS. Both sense strand derived CGG repeats and antisense strand derived CCG repeats support non-AUG initiated (RAN) translation of homopolymeric proteins in potentially 6 different reading frames. Howev...

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Literature Corpus work: 73974cb4-f037-51d7-b64d-8d8806bdaad5
DOI: 10.1101/672444

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