Mitochondrial diseases animal models

As in the case of other cardiovascular diseases, the possibility of antioxidant treatment of diabetes mellitus has been studied in both animal models and diabetic patients. The treatment of streptozotocin-induced diabetic rats with a-lipoic acid reduced superoxide production by aorta and superoxide and peroxynitrite formation by arterioles providing circulation to the region of the sciatic nerve, suppressed lipid peroxidation in serum, and improved lens glutathione level [131]. In contrast, hydroxyethyl starch desferrioxamine had no effect on the markers of oxidative stress in diabetic rats. Lipoic acid also suppressed hyperglycemia and mitochondrial superoxide generation in hearts of glucose-treated rats [132],... 

Mutations in Nuclear Genes and Animal Models of Mitochondrial Diseases... 106... 

In this chapter we review the defects of respiratory function and DNA mutations in the mitochondrial genome and nuclear DNA underlying mitochondrial diseases and discuss the roles that oxidative stress, oxidative damage, and apoptosis may play in the pathogenesis of this group of overt metabolic disorders. The cell cultures and animal models for studies of mitochondrial diseases and potential therapies are also discussed. 

Moreover, CoQi0, menadione, ascorbate, and superoxide dismutase-mimetic manganese-5,10,15,20-tetrakis (4-benzoic acid) porphyrin (MnTBAP) have been shown to mediate their antioxidant effects on the ROS and the oxidative stress involved in neuronal death in mitochondrial diseases (S4, T6). There is increasing evidence to support the role of ROS in cell death (Rl), and antioxidants appear to have benefits in animal models of mitochondrial disease (Wl). For example, Melov et al. (M9) reported that mice lacking mitochondrial MnSOD exhibited a... 

Trancikova, A., E. Tsika, and D. J. Moore. Mitochondrial dysfunction in genetic animal models of Parkinson s disease. 16(9), 2012 896-919. 

---