Modeling Myotonic Dystrophy Type 2 Using IDrosophila melanogaster/I

Myotonic dystrophy 2 (DM2) is a genetic multi-systemic disease primarily affecting skeletal muscle. It is caused by CCTGn expansion in intron 1 of the CNBP gene, which encodes a zinc finger protein. DM2 disease has been successfully modeled in Drosophila melanogaster, allowing the identification and...

Full description

Saved in:
Bibliographic Details
Published in:International journal of molecular sciences 2023-09, Vol.24 (18)
Main Authors: Marzullo, Marta, Coni, Sonia, De Simone, Assia, Canettieri, Gianluca, Ciapponi, Laura
Format: Article
Language:English
Subjects:
Analysis
DNA binding proteins
Drosophila
Genes
Health aspects
Muscles
Myotonic dystrophy
Neurophysiology
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Myotonic dystrophy 2 (DM2) is a genetic multi-systemic disease primarily affecting skeletal muscle. It is caused by CCTGn expansion in intron 1 of the CNBP gene, which encodes a zinc finger protein. DM2 disease has been successfully modeled in Drosophila melanogaster, allowing the identification and validation of new pathogenic mechanisms and potential therapeutic strategies. Here, we describe the principal tools used in Drosophila to study and dissect molecular pathways related to muscular dystrophies and summarize the main findings in DM2 pathogenesis based on DM2 Drosophila models. We also illustrate how Drosophila may be successfully used to generate a tractable animal model to identify novel genes able to affect and/or modify the pathogenic pathway and to discover new potential drugs.
ISSN:1422-0067
DOI:10.3390/ijms241814182