Morphofunctional Alterations of Mitochondria in Neurodegenerative Diseases

Mitochondria are dynamic organelles involved in many cellular functions; the best known is the production of energy, in the form of adenosine triphosphate (ATP), through the electron transport chain (ECT) and the oxidative phosphorylation pathway. Therefore, mitochondria are abundant in cells requiring high-energy source, such as neurons and muscle cells. Alterations in their morphology and function are commonly observed in different neurodegenerative diseases, including Parkinson’s disease, Amyotrophic Lateral Sclerosis, Huntington’s disease and Alzheimer’s disease. However, it is unclear whether such dysfunctions are a cause or an effect of the underlying pathology, and whether they could represent a viable therapeutic target. The purpose of this thesis is to provide insights into mitochondria dysfunctions in three neurological disorders: Alzheimer’s disease, Amyotrophic Lateral Sclerosis and Spinal Muscular Atrophy. The authors of the scientific papers that have been analysed in this thesis, demonstrated the presence of morphological alterations and malfunctions in the mitochondria in the mouse models resuming the specific pathologies.