scholarly journals Identification of Interphotoreceptor retinoid-binding protein in the Schisis cavity fluid of a patient with congenital X-linked Retinoschisis

2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Fukutaro Mano ◽  
Koji Sugioka ◽  
Kazuki Kuniyoshi ◽  
Hiroyuki Kondo ◽  
Shunji Kusaka

Abstract Background This case report describes the surgical outcome in a patient with congenital X-linked retinoschisis (CXLRS) and the results of proteomic analysis of surgically extracted samples from both vitreous and intraschisis cavities by mass spectrometry. Case presentation A 3-month-old boy presented with extensive retinoschisis involving macula and retinal periphery in both eyes. Genetic analysis confirmed retinoschisin 1 mutation (c.554C > T), and an electroretinogram showed significant reduction of b-wave and decreased cone and rod responses, which led to a diagnosis of CXLRS. By performing pars plana vitrectomy, including inner wall retinectomy, clear visual axes with stable retinal conditions and functional vision in both eyes were obtained during the 4 years of follow-up. Proteomic analysis of surgically retrieved fluid from the intraschisis cavity revealed a higher expression of interphotoreceptor retinoid-binding protein (IRBP) than that from the vitreous humor. However, both samples showed equal levels of albumin, transferrin, and pigment epithelium-derived factor. Conclusions Cellular adhesive imperfection in CXLRS may cause IRBP diffusion from the interphotoreceptor matrix, resulting in the strong expression of IRBP in the intraschisis cavity. An impaired retinoid cycle caused by an absence of IRBP in the retina may potentially underlie the pathology of CXLRS.

1985 ◽  
Vol 100 (5) ◽  
pp. 1676-1681 ◽  
Author(s):  
J G Hollyfield ◽  
H H Varner ◽  
M E Rayborn ◽  
G I Liou ◽  
C D Bridges

Between the pigment epithelium and the outer limiting membrane of the retina is an extracellular compartment filled with the interphotoreceptor matrix (IPM). A prominent component of the IPM is a glycoprotein known as interstitial retinol-binding protein (IRBP). Using in vitro techniques, we compared the ability of the cells that border this compartment to internalize colloidal gold (CG) coated with either IRBP or ovalbumin, a glycoprotein not found in the IPM. Neither IRBP-CG nor ovalbumin-CG was internalized by the Muller's cells. Both rod and cone photoreceptors take up IRBP-CG, which is observed in small vesicles and multivesicular bodies. Neither photoreceptor type takes up ovalbumin-CG. Acid phosphatase cytochemistry indicates that acid phosphatase reaction product in the multivesicular bodies co-localizes with IRBP-CG, which suggests that this molecule is degraded by rod and cone photoreceptors and is not recycled. The pigment epithelium internalizes IRBP-CG and ovalbumin-CG, both of which remain in small cytoplasmic vesicles near the apical plasma membrane. There is no indication that vesicles that contain either IRBP-CG or ovalbumin-CG fuse with the lysosomal system in the pigment epithelial cells during the incubation.


2005 ◽  
Vol 15 (2) ◽  
pp. 289-291 ◽  
Author(s):  
D. Tognetto ◽  
C. Haritoglou ◽  
A. Kampik ◽  
G. Ravalico

Purpose To describe the occurrence of massive macular edema and visual loss after indocyanine green-assisted (ICG) macular pucker surgery. Methods/Results A 74 years old female presented with a macular pucker and a hypertrophy of the retinal pigment epithelium (RPE) in her left eye. The preoperative visual acuity (VA) was 20/100. Surgery consisted of cataract extraction, lens implantation and standard pars plana vitrectomy with peeling of epiretinal tissue followed by the removal of the internal limiting membrane (ILM) remnants stained using a 0.05% ICG solution. One day after surgery, VA was counting fingers. There was an extensive macular edema and retinal thickening with hyperfluorescence during fluorescein angiography and pronounced autofluorescence using ICG filters. During follow up, the macular edema resolved completely, but VA decreased to 20/800 at six months postoperatively. There was a central scotoma and unstable fixation seen during microperimetry. Discussion This case report indicates that ICG might come into contact with bare retina if injected following removal of epiretinal membranes. Whether the observed RPE hypertrophy might have contributed to the pathogenesis of the adverse effect described remains hypothetical.


Awareness of important anatomical landmarks during vitreoretinal surgery contributes to favorable outcomes in the postoperative period. While external anatomical landmarks include pars plana, ora serrata, and vortex veins, long posterior ciliary nerves, vortex vein ampulla, and optic disc constitute the internal anatomical landmarks for vitreoretinal surgery. The forces that prevent the development of retinal detachment by keeping the retina in place are the retina pigment epithelium pump, the interphotoreceptor matrix, the presence of vitreous gel, and intraocular pressure. The main aim of the surgeon should be removing vitreous as much as possible and releasing vitreoretinal tractions which cause retinal detachment and vitreoretinal interface problems.


1991 ◽  
Vol 39 (2) ◽  
pp. 171-176 ◽  
Author(s):  
K Porrello ◽  
S P Bhat ◽  
D Bok

Interphotoreceptor retinoid binding protein (IRBP) is a soluble glycolipoprotein located between the neurosensory retina and pigment epithelium, which may serve to transport vitamin A derivatives between these tissues. The specific cell type responsible for IRBP synthesis has not been well established. To address this issue, we have examined the expression of IRBP mRNA in human and cone-dominant ground squirrel retinas by in situ hybridization. Optimal labeling and histological resolution were achieved with 35S- and 3H-labeled anti-sense riboprobes made from a human IRBP cDNA clone, and semi-thin wax-embedded retinal sections. In human retina, label was localized over the inner segments of both rod and cone photoreceptors. Quantitative analysis demonstrated a fourfold higher density of label over rod inner segments. In ground squirrel retina, labeling was found almost exclusively over the inner segments of cones. The results indicate that in human retina both rods and cones express IRBP mRNA, albeit at different levels. In cone-dominant species such as the ground squirrel, cones are the principal cell type responsible for IRBP mRNA synthesis.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Rahul A. Jonas ◽  
Chuan Chuan Wei ◽  
Jost B. Jonas ◽  
Ya Xing Wang

Abstract Background Myopic axial elongation may be due to an equatorial enlargement of Bruch’s membrane (BM), leading to a prolate eye shape and increasing strain with BM and the retinal pigment epithelium (RPE) layer at the posterior pole. The increased BM strain may cause an enlargement of Bruch’s membrane opening (BMO) of the optic nerve head, with the subsequent development and enlargement of parapapillary gamma zone as BM-free parapapillary zone. The increased strain within BM and RPE may also cause lacquer cracks (LCs) as linear breaks in the RPE and / or BM. Studies suggested that a more marked gamma zone enlargement is associated with lower prevalence of LCs or macular BM defects. Here report on the disappearance of a LC during a 10-year follow-up of a highly myopic eye, concurrent with a marked increase in gamma zone. Case presentation A 56-year-old woman showed in her right eye (axial length measured 30.69 mm) a LC, vertically oval optic disc, and parapapillary gamma zone in 2001. When re-examined in 2006, gamma zone had enlarged, while the LC was no longer detectable. In 2011, the LC was not visible neither upon ophthalmoscopy and or upon optical coherence tomography (OCT), while gamma zone had further enlarged. The gamma zone enlargement occurred in a direction perpendicular to the direction of the former LC. Conclusions The observation suggest that a LC can decrease in width, in temporal association with an enlargement of gamma zone. It fits with the notion that an enlargement of the BMO (i.e., enlarging gamma zone) may lead to a relaxation of the BM strain and subsequently to a decrease in the width of the LC.


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