Mitochondrial Neuropathology
To function, cells mostly rely on the ability of the mitochondrial respiratory chain to produce energy in the form of adenosine triphosphate, which they subsequently use to maintain their metabolic balance. Severe alterations of the mitochondrial machinery involved in energy generation lead to a group of fatal pathologies known as mitochondrial disease (MD), affecting 1:5000 births. Currently, there is no cure and the treatments available are mostly ineffective.
Mitochondrial dysfunction-mediated pathologies are characterized by restricted anatomical and cellular specificity, where select cell types show increased vulnerability for the pathological process. In this regard, high energy-requiring tissues such as the nervous system are primarily affected. However, the mechanisms leading to neuronal susceptibility to mitochondrial dysfunction are largely unknown.
Ref: 2021 SGR 00720
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