A Chlorzoxazone-Baclofen Combination Improves Cerebellar Impairment in Spinocerebellar Ataxia Type 1.

ABSTRACT:

Background

A combination of central muscle relaxants, chlorzoxazone and baclofen (chlorzoxazone-baclofen), has been proposed for treatment of cerebellar symptoms in human spinocerebellar ataxia. However, central muscle relaxants can worsen balance. The optimal dose for target engagement without toxicity remains unknown. Using the genetically precise Atxn1^154Q/2Q model of spinocerebellar ataxia type 1, we aimed to determine the role of cerebellar dysfunction in motor impairment. We also aimed to identify appropriate concentrations of chlorzoxazone-baclofen needed for target engagement without toxicity to plan for human clinical trials.

Methods

We use patch clamp electrophysiology in acute cerebellar slices and immunostaining to identify the specific ion channels ta

SUBMITTER: Bushart DD 

PROVIDER: S-EPMC7987844 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature