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Insights into dentatorubral-pallidoluysian atrophy from a new Drosophila model of disease

5 Feb 2025

Abstract excerpt

Dentatorubral-pallidoluysian atrophy (DRPLA) is a neurodegenerative disorder that presents with ataxia, dementia and epilepsy. As a member of the polyglutamine family of diseases, DRPLA is caused by abnormal CAG triplet expansion beyond 48 repeats in the protein-coding region of ATROPHIN 1 ( ATN1 ), a transcriptional co-repressor. To better understand DRPLA, we generated new Drosophila lines that can be induced...

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PMID: 39921111
DOI: 10.1016/j.nbd.2025.106834

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