A comparison study of pathological features and drug efficacy between Drosophila models of C9orf72 ALS/FTD

Lee, Davin; Jeong, Hae Chan; Kim, Seung Yeol; Chung, Jin Yong; Cho, Seok Hwan; Kim, Kyoung Ah; Cho, Jae Ho; Ko, Byung Su; Cha, In Jun; Chung, Chang Geon; Kim, Eun Seon; Lee, Sung Bae

doi:10.1016/j.mocell.2023.12.003

Molecules and Cells

2024

DOI: 10.1016/j.mocell.2023.12.003

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A comparison study of pathological features and drug efficacy between Drosophila models of C9orf72 ALS/FTD

Davin Lee,

Hae Chan Jeong,

Seung Yeol Kim

et al.

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Cited by 2 publications

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Promising animal models for amyotrophic lateral sclerosis drug discovery: a comprehensive update

Lescouzères,

Patten

2024

Expert Opinion on Drug Discovery

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Promising animal models for amyotrophic lateral sclerosis drug discovery: a comprehensive update

Lescouzères,

Patten

2024

Expert Opinion on Drug Discovery

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Decoding Nucleotide Repeat Expansion Diseases: Novel Insights from Drosophila melanogaster Studies

Atienzar-Aroca,

Kat,

López-Castel

2024

IJMS

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Drosophila melanogaster usage has provided substantial insights into the pathogenesis of several nucleotide repeat expansion diseases (NREDs), a group of genetic diseases characterized by the abnormal expansion of DNA repeats. Leveraging the genetic simplicity and manipulability of Drosophila, researchers have successfully modeled close to 15 NREDs such as Huntington’s disease (HD), several spinocerebellar ataxias (SCA), and myotonic dystrophies type 1 and 2 (DM1/DM2). These models have been instrumental in characterizing the principal associated molecular mechanisms: protein aggregation, RNA toxicity, and protein function loss, thus recapitulating key features of human disease. Used in chemical and genetic screenings, they also enable us to identify promising small molecules and genetic modifiers that mitigate the toxic effects of expanded repeats. This review summarizes the close to 150 studies performed in this area during the last seven years. The relevant highlights are the achievement of the first fly-based models for some NREDs, the incorporation of new technologies such as CRISPR for developing or evaluating transgenic flies containing repeat expanded motifs, and the evaluation of less understood toxic mechanisms in NREDs such as RAN translation. Overall, Drosophila melanogaster remains a powerful platform for research in NREDs.

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A comparison study of pathological features and drug efficacy between Drosophila models of C9orf72 ALS/FTD

Cited by 2 publications

References 54 publications

Promising animal models for amyotrophic lateral sclerosis drug discovery: a comprehensive update

Promising animal models for amyotrophic lateral sclerosis drug discovery: a comprehensive update

Decoding Nucleotide Repeat Expansion Diseases: Novel Insights from Drosophila melanogaster Studies

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