Down regulation of the expression of mitochondrial phosphopantetheinyl-proteins in pantothenate kinase-associated neurodegeneration: pathophysiological consequences and therapeutic perspectives

Down regulation of the expression of mitochondrial phosphopantetheinyl-proteins in pantothenate kinase-associated neurodegeneration: pathophysiological consequences and therapeutic perspectives

Abstract Background Neurodegeneration with brain iron accumulation (NBIA) is a group of genetic neurological disorders frequently associated with iron accumulation in the basal nuclei of the brain characterized by progressive spasticity, dystonia, muscle rigidity, neuropsychiatric symptoms, and reti...

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Main Authors:	Mónica Álvarez-Córdoba, Marta Talaverón-Rey, Irene Villalón-García, Suleva Povea-Cabello, Juan M. Suárez-Rivero, Alejandra Suárez-Carrillo, Manuel Munuera-Cabeza, Joaquín J. Salas, José A. Sánchez-Alcázar
Format:	Article
Language:	English
Published:	BMC 2021-05-01
Series:	Orphanet Journal of Rare Diseases
Subjects:	Pantothenate kinase Pantothenate kinase-associated neurodegeneration Coenzyme A Mitochondria Pantothenate Induced neurons
Online Access:	https://doi.org/10.1186/s13023-021-01823-3

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