From the Guidelines
Mitochondria are double-membrane bound, intracellular organelles present in all cells except erythrocytes, responsible for generating adenosine triphosphate (ATP) via aerobic respiration. Their primary function is to produce energy for the cell through the process of oxidative phosphorylation, which is essential for various cellular activities 1.
Key Characteristics of Mitochondria
- They have a unique double-membrane structure
- Their primary function is the generation of adenosine triphosphate (ATP)
- They host several other metabolic pathways, including the tricarboxylic acid (TCA) cycle, urea cycle, β-fatty acid oxidation, and lipid and cholesterol synthesis 1
Mitochondrial Disorders
Mitochondrial disorders refer to the group of conditions caused by impairment of the respiratory chain, which is involved in the generation of ATP by oxidative phosphorylation 1.
Clinical Implications
Understanding the structure and function of mitochondria is crucial in clinical medicine, as mitochondrial dysfunction is linked to various diseases, and impaired mitochondrial function can have significant implications for morbidity, mortality, and quality of life.
From the Research
Definition and Function of Mitochondria
- Mitochondria are the main suppliers of neuronal adenosine triphosphate and play a critical role in brain energy metabolism 2
- They serve as Ca2+ sinks and anabolic factories and are therefore essential for neuronal function and survival 2
- Mitochondria are highly dynamic organelles that modulate apoptosis, ferroptosis, and inflammasome activation 3
Role of Mitochondria in Neurodegenerative Diseases
- Mitochondrial dysfunction has been implicated in the pathogenesis of a number of neurodegenerative disorders, including Parkinson's disease, Alzheimer's disease, amyotrophic lateral sclerosis, multisystem atrophy, and progressive supranuclear palsy 4
- Impaired mitochondrial biogenesis and abnormal mitochondrial dynamics contribute to mitochondrial dysfunction in these diseases 3
- Mitochondrial involvement in Alzheimer's disease is supported by clinical features like reduced glucose and oxygen brain metabolism and by numerous microscopic and molecular findings 5
- Mitochondria have an even more remarkable role in Parkinson's disease, with several hints showing that respiratory chain activity, in particular complex I, is impaired in the disease 5
Mitochondrial Dysfunction and Disease Progression
- Dysregulation of neuronal bioenergetics is increasingly implicated in neurodegenerative disorders, particularly Parkinson's disease 2
- Mitochondrial dysfunction plays a critical role in maintaining and fostering the neurodegenerative process, although it is still unclear whether it plays a primary role in the initial stages of these diseases 5
- Recent advances in understanding the role of mitochondrial dysfunction in neurodegenerative diseases have expanded the opportunities for neurotherapeutics targeting mitochondria to alleviate symptoms and slow disease progression 6