Muscle Damage in Dystrophic mdx Mice Is Influenced by the Activity of Ca²⁺-Activated K_Ca3.1 Channels.

ABSTRACT: Duchenne muscular dystrophy (DMD) is an X-linked disease, caused by a mutant dystrophin gene, leading to muscle membrane instability, followed by muscle inflammation, infiltration of pro-inflammatory macrophages and fibrosis. The calcium-activated potassium channel type 3.1 (K_Ca3.1) plays key roles in controlling both macrophage phenotype and fibroblast proliferation, two critical contributors to muscle damage. In this work, we demonstrate that pharmacological blockade of the channel in the mdx mouse model during the early degenerative phase favors the acquisition of an anti-inflammatory phenotype by tissue macrophages and reduces collagen deposition in muscles, with a concomitant reduction of muscle damage. As already observed with other treatments, no improvement in mus

SUBMITTER: Morotti M 

PROVIDER: S-EPMC9025295 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature