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Simulate X-linked retinoschisis (XLRS) — the most common cause of juvenile-onset macular degeneration in males, with an estimated prevalence of 1 in 5,000–25,000 males worldwide. XLRS is caused by hemizygous mutations in the RS1 gene (Xp22.13), which encodes retinoschisin — a soluble secreted 224-amino acid protein produced exclusively by photoreceptors (both rods and cones) and bipolar cells. Retinoschisin is secreted as a disulphide-linked homo-octamer that acts as a critical retinal adhesion molecule, maintaining the structural and functional integrity of photoreceptor–bipolar cell synaptic connections at the outer plexiform layer (OPL), and the Müller cell–inner retinal neuron architecture at the inner nuclear layer (INL). Retinoschisin binds to the retinal Na/K-ATPase and L-type voltage-gated calcium channels on the surface of Müller cells and bipolar cell dendrites, providing the structural "glue" that maintains retinal laminar integrity. When RS1 is absent or dysfunctional, the loss of cell-cell adhesion at the INL and OPL produces the hallmark pathology: splitting (schisis) of the retinal layers — most characteristically at the macula (producing the pathognomonic spoke-wheel or stellate macular cystoid pattern visible on OCT), and in 50% of cases also at the peripheral retina (producing large schisis cavities with elevated inner retinal "veils" and inner-wall hole formation that can lead to vitreous haemorrhage or rhegmatogenous retinal detachment). The electronegative ERG (preserved a-wave with selectively reduced b-wave, reflecting inner retinal dysfunction from disrupted bipolar cell signalling) is the functional hallmark of XLRS and is present in virtually all affected males. Simulate three clinical presentations: foveal-only schisis, peripheral retinoschisis, and combined foveal + peripheral with vitreous haemorrhage complications. ΔE colour shift, CIE xy chromaticity, and image simulation.

X-linked retinoschisis colour science simulation by Auric Artisan.

Base color
Schisis pattern & settings
Schisis cavity / retinal splitting severity 50%
Image simulation
Upload JPG/PNG (max 1200 × 1200). See how a scene appears through foveal schisis (spoke-wheel cystoid macular disruption with central blur and metamorphopsia), peripheral retinoschisis (large schisis cavities producing scotomas and vitreous veil shadows), or combined presentation (extensive retinal splitting with vitreous haemorrhage producing floaters and diffuse visual loss).
Research notes
RS1 retinoschisin — unique retinal adhesion protein: Retinoschisin is the only known secreted protein containing a discoidin domain (DS domain) expressed exclusively in the retina. The DS domain mediates cell-surface binding through interaction with the α3 subunit of the retinal Na/K-ATPase (ATP1A3) and with phospholipids in the photoreceptor inner segment and bipolar cell membranes. The homo-octameric structure (eight retinoschisin monomers linked by Cys59–Cys223 disulphide bonds) creates a multivalent binding platform that physically bridges adjacent retinal cells — essentially acting as biological "spot welds" maintaining retinal laminar integrity. When RS1 is mutated, these spot welds fail → retinal layer splitting → schisis cavities. The electronegative ERG reflects disrupted signal transmission from photoreceptors to bipolar cells due to the OPL synaptic splitting.
Swatches
Normal
HEX: — • RGB: — • xy: —
XLRS affected
HEX: — • RGB: — • xy: —
ΔE (CIE76)
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ΔE (CIEDE2000)
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Deep preview
Normal
XLRS (deep)
Chromaticity (CIE xy)
Retinal schisis chromaticity shift
D65 white point: 0.313, 0.329
Image simulation

X-linked retinoschisis simulations model the inner retinal signal disruption caused by RS1 retinoschisin deficiency and retinal layer splitting in the sRGB colour space. Foveal schisis: models spoke-wheel macular cystoid disruption as central cone signal degradation — primarily affecting L/M-cone pathway fidelity through Müller cell dysfunction and INL splitting; visual acuity typically stabilises at 6/12–6/36 in first-second decade with slow deterioration thereafter. Peripheral schisis: models wider-field retinal splitting as peripheral cone and rod signal disruption — the larger OPL schisis cavities disrupt both photoreceptor-bipolar synaptic transmission and Müller cell-mediated potassium siphoning, producing broader field desaturation. Combined: models both mechanisms simultaneously with additional vitreous haemorrhage opacity factor. The electronegative ERG reflects inner retinal b-wave generation failure from disrupted bipolar cell depolarisation. ΔE2000 per Sharma et al. (2005). Not for clinical diagnosis — XLRS requires SD-OCT (foveal cystoid spaces), ERG (electronegative waveform), and RS1 genetic testing.

Multi-condition comparison
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Compare foveal schisis (spoke-wheel INL macular splitting), peripheral retinoschisis (OPL cavity with vitreous veils), and combined presentation (extensive splitting with VH/RD complications).