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Gene Expression Profiling of Mitochondrial Oxidative Phosphorylation (OXPHOS) Complex I in Friedreich Ataxia (FRDA) Patients

Friedreich ataxia (FRDA) is the most frequent progressive autosomal recessive disorder associated with unstable expansion of GAA trinucleotide repeats in the first intron of the FXN gene, which encodes for the mitochondrial frataxin protein. The number of repeats correlates with disease severity, wh...

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Detalles Bibliográficos
Autores principales: Salehi, Mohammad Hossein, Kamalidehghan, Behnam, Houshmand, Massoud, Yong Meng, Goh, Sadeghizadeh, Majid, Aryani, Omid, Nafissi, Shahriar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3976380/
https://www.ncbi.nlm.nih.gov/pubmed/24705504
http://dx.doi.org/10.1371/journal.pone.0094069