An introduction to mitochondria, their structure and functions

Abstract

Mitochondria are essential intracellular organelles whose central role in maintaining energy homeostasis places them at the heart of cell integrity, function and survival. As the reliable provision of energy is so fundamental to every aspect of cellular function, mitochondrial dysfunction inevitably has devastating implications for the cell, the tissue and the organism. This is especially critical in the nervous system, where subtle changes in signalling and function can have catastrophic global consequences. Further, as postmitotic cells are heavily dependent on oxidative phosphorylation and are morphologically enormously complex, neurons pose a unique set of challenges for the mitochondrial population that reside within them. Mitochondrial dysfunction has profound consequences for the nervous system and is implicated in a host of neurological and neurodegenerative diseases. The following chapter introduces the form and function of these fascinating organelles and introduces key concepts and vulnerabilities that may underlie their involvement in neurodegenerative conditions. This introduction lays the foundation for the following chapters, which will explore specific aspects of the roles of these organelles in a range of neurodegenerative disease.