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. Author manuscript; available in PMC: 2022 Jul 4.
Published in final edited form as: Biochim Biophys Acta Mol Basis Dis. 2020 Feb 24;1866(6):165743. doi: 10.1016/j.bbadis.2020.165743

Figure 1: LHON selectively targets retinal ganglion cells.

Figure 1:

In contrast to most other somatic cells, neurons depend highly on oxidative phosphorylation and not glycolysis for cellular ATP production under normal physiologic conditions. This makes them more vulnerable to the effects of mitochondrial dysfunction. Neurons are also more metabolically active, with the dendrites, synaptic terminals, and especially the axon being regions of high ATP demand. Retinal ganglion cells (RGCs) have a unique anatomical distinction from typical CNS neurons, however. The entire course of the axon through the retinal nerve fiber layer remains unmyelinated. Only after penetrating and passing posterior to the collagenous lamina cribrosa do oligodendrocytes myelinate RGC axons to form the optic nerve. This extensive lack of myelination makes RGCs especially demanding for a constant supply of ATP- and especially prone to injury when the supply is cut off.