9. Metabolic Changes: Fueling the Disease
Another cause of Huntington’s disease progression lies in metabolic changes within the brain. The mutated huntingtin protein interferes with energy metabolism, leading to deficits that can further drive the disease’s progression.
Neurons are highly active cells that require a lot of energy to function. In Huntington’s disease, the abnormal huntingtin protein disrupts the way neurons produce and use energy. This disruption leads to an energy deficit, contributing to neuronal dysfunction and death.
The mitochondria, often referred to as the cell’s powerhouses, are particularly affected by the huntingtin protein. Impaired mitochondrial function leads to less efficient energy production, contributing to the energy crisis in neurons.
Moreover, faulty mitochondria can also produce harmful reactive oxygen species, contributing to oxidative stress and neuronal damage. (9)
