Mouse models of non-dystrophic and dystrophic myotonia exhibit nociplastic pain-like behaviors.

ABSTRACT: Pain is a prominent and debilitating symptom in myotonic disorders, including myotonia congenita and myotonic dystrophy type 1 (DM1). Although patients frequently report chronic pain, its underlying mechanisms remain poorly defined. In both disorders, impaired chloride conductance through voltage-gated CLC-1 chloride channels in skeletal muscle disrupts membrane repolarization, leading to delayed relaxation and persistent muscle hyperexcitability. Here, we investigated the pathophysiology of pain in mouse models of acute and chronic myotonia. In the acute model, a single intraperitoneal injection of anthracene-9-carboxylic acid (9-AC, 30 mg/kg), a selective ClC-1 antagonist, induced transient muscle stiffness, cramping, and gait abnormalities, followed by prolonged pain-like behavior, incl

SUBMITTER: Nelson TS 

PROVIDER: S-EPMC11212949 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature