Khondrion, a biopharmaceutical company based in the Netherlands, has received an Innovation Credit of up to €5M from the Netherlands Enterprise Agency for its work in developing therapies for primary mitochondrial disease (PMD).

This funding, which is being matched by private investment, will support the first phase of Khondrion’s pivotal Phase 3 clinical trial for sonlicromanol. Sonlicromanol is a novel small-molecule therapy designed to target m.3243A>G PMD, the most prevalent genetic form of mitochondrial disease. The Phase 3 trial is set to commence in the second half of 2025, a significant step towards Khondrion’s goal of bringing the first approved treatment for these conditions to market.

Khondrion, founded by Jan Smeitink, focuses on developing treatments for mitochondrial diseases. Its leading pipeline candidate, KH176, is an oral small molecule currently in Phase IIb clinical development for various mitochondrial disorders. The company has obtained Orphan Drug Designation for MELAS spectrum disorders and Leigh disease in Europe, as well as for all inherited mitochondrial respiratory chain disorders in the US.

In addition to its research efforts, Khondrion collaborates with patient organizations and a global network of clinical and academic partners to accelerate the discovery and development of potential therapies for individuals with mitochondrial diseases.

Conclusion

Khondrion’s receipt of the Innovation Credit underscores the company’s commitment to advancing treatments for primary mitochondrial disease. With ongoing support and strategic partnerships, Khondrion is poised to make a meaningful impact in the field of mitochondrial medicine.

FinSMEs

06/05/2025