Propagation and Selectivity of Axonal Loss in Leber Hereditary Optic Neuropathy.

ABSTRACT: Leber hereditary optic neuropathy (LHON) is a syndrome of subacute loss of central vision associated with mutations in mitochondrial DNA coding for components of complex I. LHON preferentially involves small axons in the temporal optic nerve, but the reason is unclear. We performed a Monte Carlo simulation of the spread of injury in LHON axons to better understand the predilection for small axons. Optic nerve slices were modeled as grids containing axons with sizes from reported regional distributions. The propagation of injury from a localized concentration of superoxide was simulated as the spread via passive diffusion from one axon to adjacent axons, with basal production and scavenging rate proportional to axonal area and volume, respectively. Axonal degeneration occurred when intra-ax

SUBMITTER: Coussa RG 

PROVIDER: S-EPMC6491426 | biostudies-literature | 2019 Apr

REPOSITORIES: biostudies-literature