Expression of Myomaker and Myomerger in myofibers causes muscle pathology.

ABSTRACT:

Background

Skeletal muscle development and regeneration depend on cellular fusion of myogenic progenitors to generate multinucleated myofibers. These progenitors utilize two muscle-specific fusogens, Myomaker and Myomerger, which function by remodeling cell membranes to fuse to each other or to existing myofibers. Myomaker and Myomerger expression is restricted to differentiating progenitor cells as they are not detected in adult myofibers. However, Myomaker remains expressed in myofibers from mice with muscular dystrophy. Ablation of Myomaker from dystrophic myofibers results in reduced membrane damage, leading to a model where persistent fusogen expression in myofibers, in contrast to myoblasts, is harmful.

Methods

Dox-inducible transgenic mice were developed to ectopicall

SUBMITTER: Witcher PC 

PROVIDER: S-EPMC10150476 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature