Erratum to: Bone spicule pigment formation in retinitis pigmentosa: insights from a mouse model

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RETINAL DISORDERS Bone spicule pigment formation in retinitis pigmentosa: insights from a mouse model

Background Bone spicule pigments (BSP) are a hallmark of retinitis pigmentosa (RP). In this study, we examined the process of BSP formation in the rhodopsin knockout (rho) mouse, a murine model for human RP. Methods In rho mice from 2 to 16 months of age, representing the range from early to late stages of degeneration, retinal sections and whole mounts were examined morphologically by light an...

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A retinitis pigmentosa GTPase regulator (RPGR)-deficient mouse model for X-linked retinitis pigmentosa (RP3).

The X-linked RP3 locus codes for retinitis pigmentosa GTPase regulator (RPGR), a protein of unknown function with sequence homology to the guanine nucleotide exchange factor for Ran GTPase. We created an RPGR-deficient murine model by gene knockout. In the mutant mice, cone photoreceptors exhibit ectopic localization of cone opsins in the cell body and synapses and rod photoreceptors have a red...

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Altered proteoglycans in cultured human retinitis pigmentosa retinal pigment epithelium.

Proteoglycans are involved in a variety of cell-cell and cell-matrix interactions. These include cell adhesion, growth regulation and a number of developmental processes. Their involvement in such interactions may be of particular importance in retinitis pigmentosa (RP) because of the detachment and migration of retinal pigment epithelial (RPE) cells often associated with this condition. Becaus...

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Retinitis pigmentosa.

Hereditary degenerations of the human retina are genetically heterogeneous, with well over 100 genes implicated so far. This Seminar focuses on the subset of diseases called retinitis pigmentosa, in which patients typically lose night vision in adolescence, side vision in young adulthood, and central vision in later life because of progressive loss of rod and cone photoreceptor cells. Measures ...

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Photoaversion in retinitis pigmentosa.

Photoaversion, or light-induced interference with visual comfort and performance, has been a recognised but poorly documented symptom in retinitis pigmentosa (RP). We found that a majority of our RP patients complained of photoaversion even in the absence of significant cataract. RP patients had reduced contrast sensitivity relative to normal people, but the decrement in their visual performanc...

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ژورنال

عنوان ژورنال: Graefe's Archive for Clinical and Experimental Ophthalmology

سال: 2010

ISSN: 0721-832X,1435-702X

DOI: 10.1007/s00417-010-1357-2