Mitochondrial iron and energetic dysfunction distinguish fibroblasts and induced neurons from pantothenate kinase-associated neurodegeneration patients

Mitochondrial iron and energetic dysfunction distinguish fibroblasts and induced neurons from pantothenate kinase-associated neurodegeneration patients

Pantothenate kinase-associated neurodegeneration is an early onset autosomal recessive movement disorder caused by mutation of the pantothenate kinase-2 gene, which encodes a mitochondrial enzyme involved in coenzyme A synthesis. The disorder is characterised by high iron levels in the brain, althou...

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Bibliographic Details
Main Authors: Paolo Santambrogio, Sabrina Dusi, Michela Guaraldo, Luisa Ida Rotundo, Vania Broccoli, Barbara Garavaglia, Valeria Tiranti, Sonia Levi
Format: Article
Language:English
Published: Elsevier 2015-09-01
Series:Neurobiology of Disease
Subjects:
Iron metabolism
Mitochondria
Reactive oxygen species
NBIA
PANK2
Induced neurons
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996115000984

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http://www.sciencedirect.com/science/article/pii/S0969996115000984

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