Oxidative stress in inherited mitochondrial diseases
G Hayashi, G Cortopassi - Free Radical Biology and Medicine, 2015 - Elsevier
Mitochondria are a source of reactive oxygen species (ROS). Mitochondrial diseases are the
result of inherited defects in mitochondrially expressed genes. One potential …
result of inherited defects in mitochondrially expressed genes. One potential …
Milestones in Friedreich ataxia: more than a century and still learning
A Abrahão, JL Pedroso, P Braga-Neto… - Neurogenetics, 2015 - Springer
Friedreich ataxia (FRDA) is the most common autosomal recessive ataxia worldwide. This
review highlights the main clinical features, pathophysiological mechanisms, and …
review highlights the main clinical features, pathophysiological mechanisms, and …
[HTML][HTML] Mesenchymal stem cells improve motor functions and decrease neurodegeneration in ataxic mice
J Jones, A Estirado, C Redondo, J Pacheco-Torres… - Molecular Therapy, 2015 - cell.com
The main objective of this work is to demonstrate the feasibility of using bone marrow-
derived stem cells in treating a neurodegenerative disorder such as Friedreich's ataxia. In …
derived stem cells in treating a neurodegenerative disorder such as Friedreich's ataxia. In …
Monitoring cardiac function during idebenone therapy in Friedreich's ataxia
S Di Giovanni, P Valeria, F Bahaa… - Current Pharmaceutical …, 2015 - ingentaconnect.com
Friedreich's ataxia (FA) is associated with progressive cardiac hypertrophy resulting from a
genetic abnormality in the frataxin gene. Cardiac involvement is the most common cause of …
genetic abnormality in the frataxin gene. Cardiac involvement is the most common cause of …
Friedreich ataxia
M Pandolfo - Neuromuscular Disorders of Infancy, Childhood, and …, 2015 - Elsevier
Friedreich's ataxia (FRDA) is the most common autosomal recessive hereditary ataxia in
Caucasians. Neurological symptoms dominate the clinical picture. The underlying …
Caucasians. Neurological symptoms dominate the clinical picture. The underlying …
[PDF][PDF] Antioxidant and Therapeutic Potential of Coenzyme Q-Related Compounds
JM Villalba, MI Burón, JA González-Reyes… - Coenzyme Q10; Nova …, 2015 - researchgate.net
Coenzyme Q links basic aspects of energy metabolism, antioxidant protection and the
regulation of cell growth and death. The use of coenzyme Q10 as a therapeutic molecule in …
regulation of cell growth and death. The use of coenzyme Q10 as a therapeutic molecule in …