Friedreich Ataxia Mouse Models with Progressive Cerebellar and Sensory Ataxia Reveal Autophagic Neurodegeneration in Dorsal Root Ganglia

Friedreich ataxia (FRDA), the most common recessive ataxia, is characterized by degeneration of the large sensory neurons of the spinal cord and cardiomyopathy. It is caused by severely reduced levels of frataxin, a mitochondrial protein involved in iron-sulfur cluster (ISC) biosynthesis. Through a...

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Detalhes bibliográficos
Publicado no:J Neurosci
Main Authors: Simon, Delphine, Seznec, Hervé, Gansmuller, Anne, Carelle, Nadège, Weber, Philipp, Metzger, Daniel, Rustin, Pierre, Koenig, Michel, Puccio, Hélène
Formato: Artigo
Idioma:Inglês
Publicado em: Society for Neuroscience 2004
Assuntos:
Neurobiology of Disease
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6730414/
https://ncbi.nlm.nih.gov/pubmed/14985441
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.4549-03.2004
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