What is the reason for the visual impairment in LHON patients?
Interestingly, the eyes of patients with LHON disease function normally, meaning, unlike patients that are near or farsighted, the light becomes focused properly on the retina. However, the subsequent signal to the brain is not transmitted properly because the optic nerve is damaged. A number of genetic defects that cause LHON have been identified. All of these defects affect the power plant of our cells, the mitochondria and renders them less efficient. In fact, studies have demonstrated that mitochiondria in LHON patients have a 70% decrease in efficiency compared to mitochondria from healthy subjects.This deficit in energy production eventually leads to the death of the optic nerve cells.
