Sigma-1 Receptor is a Pharmacological Target to Promote Neuroprotection in the SOD1^G93A ALS Mice.

ABSTRACT: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder characterized by the death of motoneurons (MNs) with a poor prognosis. There is no available cure, thus, novel therapeutic targets are urgently needed. Sigma-1 receptor (Sig-1R) has been reported as a target to treat experimental models of degenerative diseases and, importantly, mutations in the Sig-1R gene cause several types of motoneuron disease (MND). In this study we compared the potential therapeutic effect of three Sig-1R ligands, the agonists PRE-084 and SA4503 and the antagonist BD1063, in the SOD1^G93A mouse model of ALS. Pharmacological administration was from 8 to 16 weeks of age, and the neuromuscular function and disease progression were evaluated using nerve conduction and rotarod tests. At the end

SUBMITTER: Gaja-Capdevila N 

PROVIDER: S-EPMC8702863 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature