Article

Ketosis rescues frataxin deficiency and corrects disease phenotypes in an FRDA animal model

2026-01-14

Abstract excerpt

Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disease caused by deficiency of the mitochondrial protein frataxin. Effective therapeutic options remain limited for FRDA. We previously demonstrated that frataxin regulates ketone body metabolism by modulating 3-Oxoacid CoA-Transferase 1 (OXCT1), the rate-limiting enzyme in ketone body catabolism. However, the mechanisms governing frataxin-depen...

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Literature Corpus work: 98e40042-3733-5512-bc45-1eec8b60a4bc
DOI: 10.64898/2026.01.14.699352

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