Sensory-motor circuit is a therapeutic target for <i>dystonia musculorum</i> mice, a model of hereditary sensory and autonomic neuropathy 6.

ABSTRACT: Mutations in Dystonin (DST), which encodes cytoskeletal linker proteins, cause hereditary sensory and autonomic neuropathy 6 (HSAN-VI) in humans and the dystonia musculorum (dt) phenotype in mice; however, the neuronal circuit underlying the HSAN-VI and dt phenotype is unresolved. dt mice exhibit dystonic movements accompanied by the simultaneous contraction of agonist and antagonist muscles and postnatal lethality. Here, we identified the sensory-motor circuit as a major causative neural circuit using a gene trap system that enables neural circuit-selective inactivation and restoration of Dst by Cre-mediated recombination. Sensory neuron-selective Dst deletion led to motor impairment, degeneration of proprioceptive sensory neurons, and disr

SUBMITTER: Yoshioka N 

PROVIDER: S-EPMC11277474 | biostudies-literature | 2024 Jul

REPOSITORIES: biostudies-literature