title: Ultrastructural features of retinal dystrophy in mutant vitiligo mice.
abstract: Ultrastructural features of the retinal pigment epithelium and photoreceptor cells were studied in vitiligo (C57BL/6 mivit/mivit) mice. Eyes from 12-day- to 56-week-old animals were analysed. Abnormal photoreceptors were seen in 12-day-old mice. By 3 weeks malformed outer segments were evident in the posterior and equatorial retina, but normal photoreceptors were present in the periphery. By 28 weeks, a marked gradient in cell loss was evident, with a progressive increase in cell viability along the posterior-peripheral axis. Viable intact photoreceptors were still present in the peripheral retina of 56-week-old mice. Melanosome content varied between adjacent pigment epithelium cells in both the posterior and peripheral retina. In the choroid, however, a steep posterior-peripheral gradient in melanosome content was evident with highest pigmentation in the periphery. In the optic nerve head region abnormal development of photoreceptors was correlated with proliferation of abnormal pigment epithelium cells. Accumulation of rod outer segment debris in the posterior and peripheral subretinal space preceded photoreceptor cell death. Short pigment epithelial microvilli without proper attachment to photoreceptors are suggestive of alterations in pigment-epithelium-photoreceptor interaction, which might affect photoreceptor differentiation and phagocytosis of rod outer segments by pigment epithelium cells.
authors.affiliation: Department of Pathology, University of Texas Health Science Center, San Antonio 78284-7750, USA.
authors.forename: I
authors.initials: I
authors.lastname: Nir
authors.forename: N
authors.initials: N
authors.lastname: Ransom
authors.forename: S B
authors.initials: SB
authors.lastname: Smith
mesh_terms: D000818:Animals; D016923:Cell Death; D002470:Cell Survival; D002829:Choroid / Q000648:ultrastructure; D008544:Melanocytes / Q000648:ultrastructure; D051379:Mice; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains; D008854:Microscopy, Electron; D008871:Microvilli / Q000648:ultrastructure; D009898:Optic Disk / Q000648:ultrastructure; D010786:Photoreceptor Cells / Q000648:ultrastructure*; D010857:Pigment Epithelium of Eye / Q000648:ultrastructure*; D012162:Retinal Degeneration / Q000473:pathology*; D013997:Time Factors; D014820:Vitiligo / Q000473:pathology*