disease 1,138 words KG: ent-dise-0270eb10 2026-03-21
kind:diseasesection:diseasesstate:published
Contents

Retinitis Pigmentosa

Disease Info
Secondary cone degenerationLoss of rods eventually leads to cone cell death
Accumulation of pigment depositsBone spicule-shaped pigment clumping in the retina
Outer retinal atrophyProgressive thinning of the outer nuclear layer
RPE dysfunctionRetinal pigment epithelium compromise
Autosomal dominant (30-40%)RHO, PRPH2, RP1, ROM1
Autosomal recessive (50-60%)USH2A, EYS, PDE6B, CNGB1
X-linked (5-15%)RPGR, RP2
Mitochondrial inheritance (rare)MT-ND4, MT-CYB
RHO (Rhodopsin)Most common autosomal dominant RP gene
USH2AUsherin protein, also causes Usher syndrome
RPGRX-linked RP, also involved in ciliary function
PDE6BCyclic GMP phosphodiesterase, rod phototransduction
DatabasesOMIMOrphanetClinicalTrialsPubMed

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