KH183

KH183 (also known as KH176m) is the primary active metabolite of sonlicromanol (KH176), an oral small molecule developed by Khondrion for the treatment of mitochondrial diseases. KH183 is formed in vivo via the biotransformation of sonlicromanol by the cytochrome P450 3A4 (CYP3A4) enzyme. It acts as a potent antioxidant and reactive oxygen species (ROS) redox modulator, targeting the oxidative stress pathways that result from mitochondrial dysfunction. As the active moiety, KH183 contributes significantly to the therapeutic effects of sonlicromanol in conditions such as Leigh's disease, MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes), and LHON (Leber's Hereditary Optic Neuropathy). Its pharmacokinetic profile was evaluated alongside the parent compound in clinical trials, including Phase 1 (NCT02544217) and subsequent Phase 2 studies.

Other names

KH176mKH-176mKH 176mactive metabolite of sonlicromanol