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Cerebellar pathology in Friedreich's ataxia: Atrophied dentate nuclei with normal iron content

BACKGROUND: In Friedreich's ataxia (FA) the genetically decreased expression of the mitochondrial protein frataxin leads to disturbance of the mitochondrial iron metabolism. Within the cerebellum the dentate nuclei (DN) are primarily affected. Histopathological studies show atrophy and accumula...

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Detalles Bibliográficos
Autores principales: Solbach, K., Kraff, O., Minnerop, M., Beck, A., Schöls, L., Gizewski, E.R., Ladd, M.E., Timmann, D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4215469/
https://www.ncbi.nlm.nih.gov/pubmed/25379420
http://dx.doi.org/10.1016/j.nicl.2014.08.018