MiM111 + CPR1

MiM111 and CPR1 (specifically CPR1-B) are second-generation small molecule mitofusin activators developed by Mitochondria in Motion in partnership with the Charcot-Marie-Tooth Research Foundation. These compounds are designed to treat Charcot-Marie-Tooth disease type 2A (CMT2A), a condition caused by mutations in the MFN2 gene that impair mitochondrial fusion and transport. MiM111 is a short-acting phenylhexanamide that induces transient mitofusin activation, while CPR1-B is a longer-acting analog providing sustained activation. Preclinical studies have demonstrated that these molecules can normalize neuromuscular dysfunction, reverse axonal and myocyte atrophy, and enhance mitochondrial trafficking within peripheral axons by targeting both Mitofusin 1 (MFN1) and Mitofusin 2 (MFN2).

Other names

N-(trans-4-Hydroxycyclohexyl)-6-phenylhexanamidephenylhexanamide mitofusin activators