Preclinical startup Mitoconix Bio has secured a $20 million series A round for its research work on neurological disorders, including its lead candidate for Huntington’s disease.

The round was led by Remiges Ventures with help from OrbiMed, Dementia Discovery Fund, Arix Bioscience, RMGP Bio-Pharma investment Fund and others.

The cash boost will be put toward the Stanford spinout’s early work on the biotech’s Huntington's disease through preclinical and clinical development, as well as expanding its pipeline into other neurodegenerative disorders, all focusing on mitochondrial function.

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Mitochondria are the energy-generating organelles within cells. These are key for human health when working properly, but excessive mitochondrial fragmentation resulting in small and dysfunctional mitochondria can cause neurodegeneration.

Its lead product, MTC-1203, is a first-in-class selective inhibitor of excessive mitochondrial division in Huntington’s disease and other neurodegenerative disorders. MTC-1203 has shown efficacy in mouse models of Huntington’s and Parkinson’s diseases, the biotech says, but of course there is a major leap from mice to humans.

“We are very excited to complete our financing round with a leading group of investors that recognize the transformative potential of Mitoconix Bio’s technology for the treatment of neurodegenerative diseases,” said Eyal Neria, CEO of Mitoconix Bio.

“The great need for medicines to slow or arrest progression of neurodegenerative diseases motivates us to rapidly advance our lead product to clinical development and to create a pipeline of therapies for these devastating diseases.”

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It was only established last August and came out of the Israel-based FutuRx incubator. Its program is based on the discoveries of its founder Prof. Daria Mochly-Rosen, the George D. Smith Professor in Translational Medicine at Stanford University, School of Medicine, and her team.

Prof. Mochly Rosen is also the founder and co-director of SPARK, the translational research program at Stanford University, and was the founder and CSO of Kai Pharmaceuticals, a peptide therapeutic company that was acquired by Amgen in 2011 for $315 million.

“The Stanford team has identified the molecular-based pathological interactions leading to excess mitochondrial fission and based on that identified pharmacological inhibitors of excess fission,” the company says. Mitoconix exclusively licensed the tech from Stanford.

Back in February, Chondrial Therapeutics made a near $23 million series A raise, led by VC Deerfield Management, for its work on rare mitochondrial diseases. This includes CTI-1601, which is being designed to treat Friedreich’s ataxia.