Mitoconix Bio

Company profile

Mitoconix Bio company profile

Mitoconix Bio is pioneering a disease-modifying strategy of improving mitochondrial functions for treating neurodegenerative diseases. Mitochondria generates ATP, the energy source for all cellular activities. Mitochondria are also involved in other essential cellular functions, including calcium sequestration, amino acid and lipid metabolism, keeping balanced redox potential and orchestrating programmed cell death. In neurons in particular, mitochondria are very dynamic organelles that constantly undergo fission (division) and fusion. This process, called mitochondrial dynamics, is critical in maintaining healthy and functional mitochondria under both normal conditions and in response to stress and in ensuring having viable mitochondria along the axons and in the synaptic boutons. A defect in either fusion or fission limits mitochondrial motility, decreases energy production, increases oxidative stress and activates pro-apoptotic signaling thereby promoting cell dysfunction and death. Mitoconix’ lead drug is a first-in-class inhibitor of pathological mitochondrial fragmentation and dysfunction with demonstrated in vivo efficacy in animal models of Huntington’s (HD) and Parkinson’s diseases (PD) and beneficial activity in patient-derived cells of HD, sporadic and genetic PD, and sporadic and genetic Alzheimer’s disease (AD).