Bimoclomol and Arimoclomol: HSP-co-Inducers for the Treatment of ProteinMisfolding Disorders, Neuropathy and Neuropathic Pain

Bimoclomol and arimoclomol are small new chemical entities which have been synthesized during the end of last century by a Hungarian pharmaceutical company, Biorex. Both compounds consistently increase Heat Shock Protein (HSP) expression and reduce functional as well as structural pathology in a series of animal models. The development of bimoclomol stagnated, most probably due the short half life time of the compound. Arimoclomol is currently in development for orphan disorders such as amyotrophic lateral sclerosis, the sphingolipidose Niemann- Pick type C and inclusion body myositis. Arimoclomol has high oral availability and good CNS penetration, without inducing troublesome CNS side effects. It might also be a promising compound for the treatment of diabetic neuropathy and neuropathic pain in (diabetic) neuropathy, due to its neuroprotective and analgesic properties.