Mitochondrial Diseases are rare progressive, multi-system, often early fatal disorders
affecting both children and adults. KH176 is a novel chemical entity currently under
development for the treatment of inherited mitochondrial diseases, including MELAS
(Mitochondrial Encephalomyopathy, Lactic acidosis, and Stroke-like episodes), Leigh's Disease
and Leber's Hereditary Optic Neuropathy (LHON). KH176 is a potent intracellular redox
modulating agent targeting the reactive oxygen species which are important in the
pathogenesis of disorders of mitochondrial oxidative phosphorylation. After demonstrating a
favourable safety profile in the pre-clinical testing, the safety, tolerability and
pharmacokinetic and pharmacodynamic characteristics of the compound will now be evaluated in
healthy male subjects in this trial