Studying the Effect of MBNL1 and MBNL2 Loss in Skeletal Muscle Regeneration.

ABSTRACT: Loss of function of members of the muscleblind-like (MBNL) family of RNA binding proteins has been shown to play a key role in the spliceopathy of RNA toxicity in myotonic dystrophy type 1 (DM1), the most common muscular dystrophy affecting adults and children. MBNL1 and MBNL2 are the most abundantly expressed members in skeletal muscle. A key aspect of DM1 is poor muscle regeneration and repair, leading to dystrophy. We used a BaCl₂-induced damage model of muscle injury to study regeneration and effects on skeletal muscle satellite cells (MuSCs) in Mbnl1^∆E3/∆E3 and Mbnl2^∆E2/∆E2 knockout mice. Similar experiments have previously shown deleterious effects on these parameters in mouse models of RNA toxicity. M

SUBMITTER: Yadava RS 

PROVIDER: S-EPMC10931579 | biostudies-literature | 2024 Feb

REPOSITORIES: biostudies-literature