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- W2059793002 endingPage "91" @default.
- W2059793002 startingPage "76" @default.
- W2059793002 abstract "Mitochondrial disorders are the most common inborn errors of metabolism affecting the oxidative phosphorylation system (OXPHOS). Because of the poor knowledge of the pathogenic mechanisms, a cure for these disorders is still unavailable and all the treatments currently in use are supportive more than curative. Therefore, in the past decade a great variety of mouse models have been developed to assess the in vivo function of several mitochondrial proteins involved in human diseases. Due to the genetic and physiological similarity to humans, mice represent reliable models to study the pathogenic mechanisms of mitochondrial disorders and are precious to test new therapeutic approaches. Here we summarize the features of several mouse models of mitochondrial diseases directly related to defects in subunits of the OXPHOS complexes or in assembly factors. We discuss how these models recapitulate many human conditions and how they have contributed to the understanding of mitochondrial function in health and disease." @default.
- W2059793002 created "2016-06-24" @default.
- W2059793002 creator A5008039801 @default.
- W2059793002 creator A5036009781 @default.
- W2059793002 creator A5056609149 @default.
- W2059793002 creator A5084495672 @default.
- W2059793002 date "2015-03-01" @default.
- W2059793002 modified "2023-10-18" @default.
- W2059793002 title "Mitochondrial Diseases Part I: Mouse models of OXPHOS deficiencies caused by defects in respiratory complex subunits or assembly factors" @default.
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