From the Guidelines
Mitochondria are the primary site of energy production in cells, generating adenosine triphosphate (ATP) via aerobic respiration, and also host several other metabolic pathways, including the tricarboxylic acid (TCA) cycle, urea cycle, β-fatty acid oxidation, and lipid and cholesterol synthesis. The functions of mitochondria can be broken down into several key areas, including:
- Energy production: Mitochondria are responsible for generating most of the energy that cells need to function, in the form of ATP, through the process of cellular respiration 1.
- Metabolic pathways: Mitochondria are involved in a range of metabolic pathways, including the TCA cycle, urea cycle, β-fatty acid oxidation, and lipid and cholesterol synthesis, as noted in a study published in the British Journal of Anaesthesia 1.
- Regulation of cellular metabolism: Mitochondria play a crucial role in regulating cellular metabolism, including maintaining calcium homeostasis and controlling programmed cell death (apoptosis).
- Cell signaling: Mitochondria are also involved in cell signaling pathways, and contain their own DNA (mtDNA) which can replicate independently within cells. The importance of mitochondrial function is highlighted by the fact that mitochondrial dysfunction is linked to numerous diseases, including neurodegenerative disorders, metabolic conditions, and aging-related pathologies, as discussed in the context of mitochondrial disorders and general anesthesia 1.
From the Research
Functions of Mitochondria
The primary functions of mitochondria include:
- Generating energy in the form of adenosine triphosphate (ATP) through oxidative phosphorylation (OXPHOS) 2, 3, 4, 5
- Storing calcium for cell signaling activities 2
- Generating heat 2
- Harbor pathways of intermediate metabolism 2
- Mediating cell growth and death 2
- Responding to oxidative stress and apoptosis 3
- Synthesizing key molecules 3
Mitochondrial Dysfunction and Disease
Mitochondrial dysfunction can lead to various diseases, including:
- Primary mitochondrial diseases (MDs) caused by mutations in mitochondrial DNA (mtDNA) or nuclear DNA genes 2, 5
- Neurodegenerative diseases such as Parkinson's disease, Alzheimer's disease, Huntington's disease, and amyotrophic lateral sclerosis (ALS) 3
- Mitochondrial myopathies characterized by early fatigue and/or fixed muscle weakness 6
Mitochondrial Structure and Function
Mitochondria are double-membrane-bound organelles present in all nucleated eukaryotic cells, with their function under dual genetic control: