Article
Fibroblasts and hiPS-Derived Astrocytes From CoPAN Patients Showed Different Levels of Iron Overload Correlated With Senescent Phenotype.
Glia - 1 Jul 2025
Cozzi Anna, Santambrogio Paolo, Moro Andrea Stefano, Pelagatti Alessio, Rubio Alicia, Balestrucci Chiara, Di Meo Ivano, Tiranti Valeria, Levi Sonia
Abstract excerpt
COASY protein-associated neurodegeneration (CoPAN) is a rare autosomal recessive disorder within the Neurodegeneration with Brain Iron Accumulation spectrum, resulting from mutations in COASY. This gene encodes the bifunctional enzyme essential for the final steps of coenzyme A biosynthesis. To elucidate the pathophysiology and iron dyshomeostasis underlying CoPAN, we analyzed fibroblasts and human induced...
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