Oxidative stress and altered lipid metabolism in Friedreich ataxia.
Friedreich ataxia is a genetic disease caused by the deficiency of frataxin, a mitochondrial protein. Frataxin deficiency impacts in the cell physiology at several levels. One of them is oxidative stress with consequences in terms of protein dysfunctions and metabolic alterations. Among others, alterations in lipid metabolism have been observed in several models of the disease. In this review we summarize the current knowledge of the molecular basis of the disease, the relevance of oxidative stress and the therapeutic strategies based on reduction of mitochondrial reactive oxygen species production. Finally, we will focus the interest in alterations of lipid metabolism as a consequence of mitochondrial dysfunction and describe the therapeutic approaches based on targeting lipid metabolism.
Departament de Ciències Mèdiques Bàsiques, IRB-Lleida, Universitat de Lleida, Lleida, Spain.
Jordi
J
Tamarit
Departament de Ciències Mèdiques Bàsiques, IRB-Lleida, Universitat de Lleida, Lleida, Spain.
Èlia
È
Obis
Departament de Ciències Mèdiques Bàsiques, IRB-Lleida, Universitat de Lleida, Lleida, Spain. Electronic address: joaquim.ros@cmb.udl.cat.
Joaquim
J
Ros
D000818:Animals; D004195:Disease Models, Animal; D005621:Friedreich Ataxia / Q000378:metabolism*; D006801:Humans; D050356:Lipid Metabolism; D008928:Mitochondria / Q000378:metabolism*; D018384:Oxidative Stress; D017382:Reactive Oxygen Species / Q000378:metabolism*
D017382:Reactive Oxygen Species
Friedreich ataxia; Iron; Lipid metabolism; Mitochondria; Oxidative stress