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Cardiomyopathy of Friedreich’s Ataxia: Use of Mouse Models to Understand Human Disease and Guide Therapeutic Development

Friedreich’s ataxia is a multisystem disorder of mitochondrial function affecting primarily the heart and brain. Patients experience a severe cardiomyopathy that can progress to heart failure and death. Although the gene defect is known, the precise function of the deficient mitochondrial protein, f...

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Detalhes bibliográficos
Main Authors: Payne, R. Mark, Pride, P. Melanie, Babbey, Clifford M.
Formato: Artigo
Idioma:Inglês
Publicado em: 2011
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3097037/
https://ncbi.nlm.nih.gov/pubmed/21360265
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00246-011-9943-6
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