Autophagy activation by urolithin-a derivative UA-36 mitigates Friedreich's ataxia pathologies induced by frataxin deficiency - PubMed
3 hours ago
- #Friedreich's ataxia
- #mitochondrial dysfunction
- #autophagy
- UA-36, a derivative of urolithin A, activates autophagy and mitigates pathologies in Friedreich's ataxia models.
- In cellular models, UA-36 restored frataxin levels, enhanced autophagy, improved mitochondrial function, and reduced oxidative stress.
- In YG8R mice, oral UA-36 improved motor coordination, gait, muscle strength, and protected against cerebellar and cardiac damage.
- Proteomic analysis showed UA-36 upregulates autophagy, mitochondrial biogenesis, and redox pathways while suppressing apoptosis and inflammation.
- The study suggests UA-36 as a promising therapeutic compound for Friedreich's ataxia by enhancing autophagy and mitochondrial quality control.