The mitochondrial genome encodes core subunits of the respiratory chain that drives oxidative phosphorylation and is, therefore, essential for energy conversion. Advances in high throughput sequencing technologies and cryoelectron microscopy have shed light on the structure and organization of the mitochondrial genome and revealed unique mechanisms of mitochondrial gene regulation. New animal models of impaired mitochondrial protein synthesis have shown how the coordinated regulation of the cytoplasmic and mitochondrial translation machineries ensures the correct assembly of the respiratory chain complexes. These new technologies and disease models are providing a deeper understanding of mitochondrial genome organization and expression and of the diseases caused by impaired energy conversion, including mitochondrial, neurodegenerative, cardiovascular, and metabolic diseases. They also provide avenues for the development of treatments for these conditions.

Instructions and Rubric:

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Format: (2 pages, including Figures and references)

Background: the structure and organization of the mitochondrial genome (1 point)

Discussion: Elaborate on technologies allowing to unravel of unique mechanisms of mitochondrial gene regulation (1.5 points)

Conclusion: Debate on disease models providing a deeper understanding of mitochondrial genome organization and expression and of the diseases caused by impaired energy conversion, including mitochondrial, neurodegenerative, cardiovascular and metabolic diseases. Create hypothesis development of treatments based on the above mentioned models ( 2 points)