HSF1 and amyotrophic lateral sclerosis: In a rat sciatic nerve crush model, treatment with BRX-220 increased motor neuron survival.24 Arimoclomol also improved motor neuron survival in both the SODG93A mouse model of amyotrophic lateral sclerosis (ALS)25, 26 and a mouse model of spinal bulbar muscular atrophy, a CAG-repeat disorder.27 Bimoclomol and its analogues BRX-220 and arimoclomol have been shown to prolong and increase the activation of HSF1,25, 28 which leads to increased upregulation of Hsp70 and Hsp90, in vitro21 and in vivo.