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Simulate Leber hereditary optic neuropathy (LHON) arising from mitochondrial DNA point mutations that cause selective degeneration of retinal ganglion cells and the papillomacular bundle. Explore central scotoma and cecocentral scotoma patterns, progressive contrast attenuation, and acquired dyschromatopsia. Inspect ΔE color shift and chromaticity deviation. Advanced neuro-ophthalmology research tool covering mtDNA mutations (m.11778G>A, m.3460G>A, m.14484T>C), Complex I electron transport chain pathophysiology, incomplete penetrance mechanisms, and current gene therapy landscape.

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Base color
Model & settings
Vision loss severity50%
Image simulation
Upload JPG/PNG (max 1200×1200). Simulated canvas next to original.
Research notes
LHON results from point mutations in mitochondrial DNA encoding Complex I (NADH dehydrogenase) subunits. The three primary mutations — m.11778G>A (ND4, ~70%), m.3460G>A (ND1, ~13%), and m.14484T>C (ND6, ~14%) — impair the electron transport chain in retinal ganglion cells, whose long unmyelinated axons are highly metabolically vulnerable. Gradual scotoma edge simulates the expanding lesion margin during the subacute conversion phase. Dyschromatopsia mode desaturates colors along the red-green axis, a characteristic LHON feature.
Swatches — Reference vs LHON Central Scotoma
Reference
HEX: — • RGB: — • xy: —
LHON Sim
HEX: — • RGB: — • xy: —
ΔE (CIE76)
ΔE (CIEDE2000)
Deep preview
Reference
Simulated
Chromaticity (CIE xy)
Achromatic axis (to D65)
D65 white point: 0.313, 0.329
Image simulation
Multi-condition comparison
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Compare normal, Condition A and Condition B across multiple severities. If an image is loaded it will be processed into the grid, otherwise color swatches are shown.