Arimoclomol maleate (BRX-220) is an orally active co-inducer of heat shock proteins ( HSP ). Arimoclomol protects motor neurons by enhancing Hsp expression, thus directly affecting protein aggregation and clearance of misfolded assemblies via the proteasome-ubiquitin system.Arimoclomol maleate can be used for the study of Niemann–Pick disease type C.