The Role of Circular-Utrophin RNAs for Muscle Sparing in DMD
Abstract
The goal of this project is to investigate the role of circular RNA of utrophin genes, which likely explains the difference of extraocular muscle phenotypes between two dystrophin/utrophin dKO mice and as the sparing mechanism of extraocular muscles in Duchenne Muscular Dystrophy (DMD). We identified 3 species of circular utrophin RNAs in extraocular muscles. We examined the role of RNA-binding proteins, such as TIA-1 and TIAR, to stabilize the circular utrophin RNA containing Exon 7 (Exon 4-8 junction) using si-RNA transfection. We established the protocol of extraocular myofiber isolation to test membrane fragility and response to calcium overload, which explains the spared muscle membrane property. To mimic the increased expression of circular-utrophin RNAs in extraocular muscles, we developed a plasmid to overexpress circular-utrophin Ex 7.Lastly, we confirmed the overexpression of circular-utrophin Ex7 in myoblasts in vitro and in FDB muscles in vivo using electroporation.
Document Details
- Document Type
- Technical Report
- Publication Date
- Oct 01, 2023
- Accession Number
- AD1219923
Entities
People
- Hyojung Choo
Organizations
- Emory University