Mitochondrial BiogenesisProcesses, Regulation, Functions and Disease

Launched October 2007 Updated May 2018 29 lectures
Prof. David Hood
York University, Canada
Summary

Mitochondria are the cellular organelles responsible for converting metabolic fuels like glucose and fatty acids into energy for the cell (ATP) via oxidative phosphorylation. The mitochondrial content of the cell corresponds closely to the functional energy requirements of a given tissue. For example, the heart, which contracts constantly has the... read morehighest mitochondrial content of all mammalian tissues, whereas type IIx muscle fibers, which are used only occasionally, have a low mitochondrial volume per fiber.

Although the primary role of mitochondria is ATP provision, mitochondria are now recognized to be involved in many other processes that are important for cellular function. These include calcium (Ca2+) homeostasis, intracellular signal transduction, and the regulation of apoptosis. In addition, a number of disease states are currently recognized to be a direct consequence of mitochondrial dysfunction.

In patients with mitochondrial disease, organelle function is impaired due to a wide variety of pathogenic mutations with origins in either mitochondrial DNA (mtDNA) or nuclear DNA. This leads to dysfunctional gene products within the electron transport chain which ultimately compromise ATP provision, resulting in reduced tissue function, impaired work capacity and exercise intolerance.

The aim of this series of talks is describe the normal processes of mitochondrial biogenesis, as well as its regulation, signaling functions, role in apoptosis and how this process could be potentially altered in aging and a variety of diseases.