Inhibition of HuR/ELAVL-1 attenuates fibrotic progression in Mdx mice with dilated cardiomyopathy.

ABSTRACT: Duchenne muscular dystrophy (DMD) arises from dystrophin deficiency, a crucial component of the dystrophin-glycoprotein complex (DGC) essential for maintaining cellular structural integrity by linking intracellular actin filaments to the basal lamina. Dysfunctions within this complex, coupled with increased inflammatory immune cell infiltration, contribute to the onset of dilated cardiomyopathy (DCM). This cardiac condition, characterized by necrosis and fibrosis, significantly impairs left ventricular function. Despite various treatment approaches, reliable effects on these pathogenic mechanisms remain elusive. RNA-binding proteins play pivotal roles in modulating pathways often dysregulated in cardiac pathology. Notably, HuR, which is upregulated in fibrotic cardiac regions and modulates

SUBMITTER: Farini A 

PROVIDER: S-EPMC12799888 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature