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Khondrion receives €2.3M funding from EU Horizon 2020 SME instrument

Khondrion was awarded €2.3M from the highly competitive EU Horizon 2020 SME instrument program. The grant will be used for the execution of a phase 2b dose-finding clinical trial with KH176 in adults with mitochondrial disease and the delivery of the milestones allowing for financing of the pivotal Phase 3 clinical trial for market approval.


Khondrion’s project KHON2bTreat “Late Clinical Development of KH176: an innovative orphan drug to reach mitochondrial disease patients & market” is one of the 65 granted projects selected out 1664 proposals.

Jan Smeitink, Khondrion’s CEO, said “This H2020 SME instrument grant is a great opportunity for Khondrion enabling us to accelerate the clinical development of KH176 for patients suffering from mitochondrial disease who have an unmet medical need for clinical relevant treatments. We are extremely proud to be awarded with the most competitive grant for SME’s in Europe and are eager to start and bring this innovative project to a success.”


KH176 is an orally bio-available small molecule in development by Khondrion for the treatment of mitochondrial (-related) diseases. The compound is a member of a new class of potential Khondrion drugs essential for the control of oxidative and redox alterations. Khondrion reported earlier that KH176 was well tolerated, appeared safe and provided first evidence of efficacy in the KHENERGY study, a Phase II exploratory trial with oral KH176 in the m.3243A>G multisystem mitochondrial MELAS and MIDD syndromes and mixed phenotypes.