Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation

Friedreich ataxia (FRDA) is the most common recessive ataxia in the Caucasian population and is characterized by a mixed spinocerebellar and sensory ataxia frequently associating cardiomyopathy. The disease results from decreased expression of the FXN gene coding for the mitochondrial protein fratax...

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Detalles Bibliográficos
Autores principales: Martelli, Alain, Puccio, Hélène
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2014
Materias:
Pharmacology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4042101/
https://www.ncbi.nlm.nih.gov/pubmed/24917819
http://dx.doi.org/10.3389/fphar.2014.00130

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4042101/
https://www.ncbi.nlm.nih.gov/pubmed/24917819
http://dx.doi.org/10.3389/fphar.2014.00130

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