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Article

CAG repeat-binding small molecule improves motor coordination impairment in a mouse model of Dentatorubral-pallidoluysian atrophy.

Neurobiology of disease - 1 Feb 2022

Hasuike Yuhei, Tanaka Hana, Gall-Duncan Terence, Mehkary Mustafa, Nakatani Kazuhiko, Pearson Christopher E, Tsuji Shoji, Mochizuki Hideki, Nakamori Masayuki

Abstract excerpt

Dentatorubral-pallidoluysian atrophy (DRPLA) is a devastating genetic disease presenting myoclonus, epilepsy, ataxia, and dementia. DRPLA is caused by the expansion of a CAG repeat in the ATN1 gene. Aggregation of the polyglutamine-expanded ATN1 protein causes neuro-degeneration of the dentatorubral and pallidoluysian systems. The expanded CAG repeats are unstable, and ongoing repeat expansions contribute to...

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PMID: 34968706
DOI: 10.1016/j.nbd.2021.105604

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