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Alpha-lipoic acid supplementation corrects pathological alterations in cellular models of pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels

Abstract Background Neurodegeneration with brain iron accumulation (NBIA) disorders are a group of neurodegenerative diseases that have in common the accumulation of iron in the basal nuclei of the brain which are essential components of the extrapyramidal system. Frequent symptoms are progressive s...

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Principais autores: Marta Talaverón-Rey, Mónica Álvarez-Córdoba, Irene Villalón-García, Suleva Povea-Cabello, Juan M. Suárez-Rivero, David Gómez-Fernández, Ana Romero-González, Alejandra Suárez-Carrillo, Manuel Munuera-Cabeza, Paula Cilleros-Holgado, Diana Reche-López, Rocío Piñero-Pérez, José A. Sánchez-Alcázar
Formato: Artigo
Idioma:Inglês
Publicado: BMC 2023-04-01
Series:Orphanet Journal of Rare Diseases
Assuntos:
Pantothenate kinase
PANK2
Pantothenate kinase-associated neurodegeneration
PKAN
Coenzyme A
Mitochondria
Acceso en liña:https://doi.org/10.1186/s13023-023-02687-5
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