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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Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:J Neurosci
Prif Awduron: Simon, Delphine, Seznec, Hervé, Gansmuller, Anne, Carelle, Nadège, Weber, Philipp, Metzger, Daniel, Rustin, Pierre, Koenig, Michel, Puccio, Hélène
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: Society for Neuroscience 2004
Pynciau:
Neurobiology of Disease
Mynediad Ar-lein: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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