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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...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2014
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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 |