Apparent Opportunities and Hidden Pitfalls: The Conflicting Results of Restoring NRF2-Regulated Redox Metabolism in Friedreich’s Ataxia Pre-Clinical Models and Clinical Trials

Friedreich’s ataxia (FRDA) is an autosomal, recessive, inherited neurodegenerative disease caused by the loss of activity of the mitochondrial protein frataxin (FXN), which primarily affects dorsal root ganglia, cerebellum, and spinal cord neurons. The genetic defect consists of the trinucleotide GA...

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Detalles Bibliográficos
Autores principales: Tiberi, Jessica, Segatto, Marco, Fiorenza, Maria Teresa, La Rosa, Piergiorgio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Review
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10215444/
https://www.ncbi.nlm.nih.gov/pubmed/37238963
http://dx.doi.org/10.3390/biomedicines11051293

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10215444/
https://www.ncbi.nlm.nih.gov/pubmed/37238963
http://dx.doi.org/10.3390/biomedicines11051293

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