Mitochondrial degradation, autophagy and neurodegenerative disease

Abstract

Mitochondria are essential for cellular and organismal health, such that mitochondrial dysfunction can contribute to ageing and age-related diseases. In particular, impairment of mitochondrial function has been shown to be an underlying cause of several human neurodegenerative disorders, including Alzheimer's disease and Parkinson's disease. In this chapter, we outline the current understanding of various mechanisms of mitochondrial quality control with a specific focus on mitophagy, the process of mitochondrial degradation via the autophagy pathway. We describe the autophagy and mitophagy pathways and highlight the key molecular players controlling these processes. Additionally, we discuss how mutations in the components of the molecular machinery controlling mitophagy can lead to the loss of neuronal function and viability and eventually result in disease. Studies of these pathways not only produce an important insight into the mechanisms of neurodegenerative diseases but also suggest molecular components of mitochondrial quality control which could be used as targets for therapeutic interventions.