Displaying publications 1 - 20 of 94 in total

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  1. Aliahmad B, Kumar DK, Hao H, Unnikrishnan P, Che Azemin MZ, Kawasaki R, et al.
    ScientificWorldJournal, 2014;2014:467462.
    PMID: 25485298 DOI: 10.1155/2014/467462
    Fractal dimensions (FDs) are frequently used for summarizing the complexity of retinal vascular. However, previous techniques on this topic were not zone specific. A new methodology to measure FD of a specific zone in retinal images has been developed and tested as a marker for stroke prediction. Higuchi's fractal dimension was measured in circumferential direction (FDC) with respect to optic disk (OD), in three concentric regions between OD boundary and 1.5 OD diameter from its margin. The significance of its association with future episode of stroke event was tested using the Blue Mountain Eye Study (BMES) database and compared against spectrum fractal dimension (SFD) and box-counting (BC) dimension. Kruskal-Wallis analysis revealed FDC as a better predictor of stroke (H = 5.80, P = 0.016, α = 0.05) compared with SFD (H = 0.51, P = 0.475, α = 0.05) and BC (H = 0.41, P = 0.520, α = 0.05) with overall lower median value for the cases compared to the control group. This work has shown that there is a significant association between zone specific FDC of eye fundus images with future episode of stroke while this difference is not significant when other FD methods are employed.
    Matched MeSH terms: Retina/pathology*; Retinal Vessels/pathology
  2. Sherina, Q., Rosiah, M., Mushawiahti, M.
    Medicine & Health, 2019;14(2):271-277.
    MyJurnal
    Acute retinal necrosis (ARN) is a rare, blinding disease that typically affects adults. However, in this case report, we highlight the diagnosis, management and outcome of herpes simplex acute retinal necrosis in a 13-year-old healthy girl, who presented with painful right eye, redness and blurring of vision for one week. Examination of the right eye showed features of granulomatous panuveitis. Optic disc was swollen and retina appeared pale. There were multiple patches of retinitis and haemorrhages at mid-periphery of the fundus with inferior serous detachment observed. Rapidly progressive inflammation in just four days along with secondary cataract that obscured fundus view, imposed greater challenge to the diagnosis and management. Intravenous acyclovir 300mg, 3 times a day was initiated promptly while vitreous fluid was sent for polymerase chain reaction, which identified Herpes Simplex Virus-1. Inflammation improved, but she developed vitreous haemorrhage secondary to proliferative retinopathy, which required panretinal photocoagulation. ARN is therefore, principally a clinical diagnosis and high index of suspicion is crucial particularly, in children for prompt diagnosis and treatment. Complications should also be addressed timely to improve the chances of preserving vision.

    Matched MeSH terms: Retina; Retinal Necrosis Syndrome, Acute
  3. Aibinu AM, Iqbal MI, Shafie AA, Salami MJ, Nilsson M
    Comput Biol Med, 2010 Jan;40(1):81-9.
    PMID: 20022595 DOI: 10.1016/j.compbiomed.2009.11.004
    The use of vascular intersection aberration as one of the signs when monitoring and diagnosing diabetic retinopathy from retina fundus images (FIs) has been widely reported in the literature. In this paper, a new hybrid approach called the combined cross-point number (CCN) method able to detect the vascular bifurcation and intersection points in FIs is proposed. The CCN method makes use of two vascular intersection detection techniques, namely the modified cross-point number (MCN) method and the simple cross-point number (SCN) method. Our proposed approach was tested on images obtained from two different and publicly available fundus image databases. The results show a very high precision, accuracy, sensitivity and low false rate in detecting both bifurcation and crossover points compared with both the MCN and the SCN methods.
    Matched MeSH terms: Retina/pathology*
  4. Norhani Mohidin, Shaznida Ghulam, Rokiah Omar
    MyJurnal
    Elektroretinogram paten (pERG) adalah pengrekodan respons retina terhadap stimulus paten yang dipancarkan bersilih ganti. Ia memberi maklumat mengenai integriti sel dalaman retina khasnya sel ganglion. Pengrekodan pERG dalam sesebuah makmal boleh dipengaruhi oleh beberapa faktor, maka piawai makmal perlu ada untuk memastikan bacaan pERG yang diperolehi boleh diulang dan dihasilkan semula. Objektif kajian ini ialah untuk menentusahkan faktor yang mungkin mempengaruhi pengukuran pERG untuk paiwaian Makmal Elektrofisiologi Jabatan Optometri, Fakulti Sains Kesihatan (FSK), Universiti Kebangsaan Malaysia (UKM). Kajian ini melibatkan 45 orang subjek yang berumur di antara 20 hingga 25 tahun yang dibahagikan kepada 3 kumpulan. Faktor yang dikaji adalah 1) kesan penggunaan anestetik Alcaine 0.5%, 2) variasi pengukuran pada waktu pagi dan petang dan 3) saiz dan bentuk target fiksasi yang berbeza terhadap bacaan pERG (amplitud dan tempoh pendam). Ujian-t berpasangan mendapati tiada perbezaan yang signifikan antara pengukuran sebelum dan selepas penggunaan Alcaine 0.5% bagi amplitud (p = 0.116) dan tempoh pendam
    (p = 0.557). Pengukuran pada waktu pagi dan petang juga menunjukkan tiada perbezaan signifikan bagi amplitud (p = 0.864) dan tempoh pendam (p = 0.174). Untuk bentuk dan saiz target yang berbeza, didapati tiada perbezaan yang signifikan untuk parameter amplitud (p = 0.125) dan tempoh pendam (p = 0.404). Kesimpulannya, penggunaan Alcaine 0.5%, pengukuran pada waktu pagi dan petang dan target fiksasi yang berbeza tidak mempengaruhi bacaan pERG di Makmal Elektrofisiologi, FSK, UKM. Hasil kajian boleh diguna pakai untuk perbandingan dalam penyelidikan ataupun tujuan pendiagnosan penyakit retina di masa hadapan.


    Matched MeSH terms: Retina; Retinal Ganglion Cells
  5. Neoh YL, Neoh PF, Salleh A, Yusof ZB, Gurusamy B, Ahmad Tajudin LS
    Ann Acad Med Singap, 2018 06;47(6):226-229.
    PMID: 30019068
    Matched MeSH terms: Retina/pathology*
  6. Chaikitmongkol V, Sagong M, Lai TYY, Tan GSW, Ngah NF, Ohji M, et al.
    Asia Pac J Ophthalmol (Phila), 2021 Nov 24;10(6):507-518.
    PMID: 34839342 DOI: 10.1097/APO.0000000000000445
    PURPOSE: Review and provide consensus recommendations on use of treat-and-extend (T&E) regimens for neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV) management with relevance for clinicians in the Asia-Pacific region.

    METHODS: A systematic search of MEDLINE, EMBASE, and Cochrane databases, and abstract databases of the Asia-Pacific Vitreo-retina Society, European Society of Retina Specialists, American Academy of Ophthalmology, and Controversies in Ophthalmology: Asia-Australia congresses, was conducted to assess evidence for T&E regimens in nAMD. Only studies with ≥100 study eyes were included. An expert panel reviewed the results and key factors potentially influencing the use of T&E regimens in nAMD and PCV, and subsequently formed consensus recommendations for their application in the Asia-Pacific region.

    RESULTS: Twenty-seven studies were included. Studies demonstrated that T&E regimens with aflibercept, ranibizumab, or bevacizumab in nAMD, and with aflibercept in PCV, were efficacious and safe. The recommendation for T&E is, after ≥3 consecutive monthly loading doses, treatment intervals can be extended by 2 to 4 weeks up to 12 to 16 weeks. When disease activity recurs, the recommendation is to reinject and shorten intervals by 2 to 4 weeks until fluid resolution, after which treatment intervals can again be extended. Intraretinal fluid should be treated until resolved; however, persistent minimal subretinal fluid after consecutive treatments may be tolerated with treatment intervals maintained or extended if the clinical condition is stable.

    CONCLUSIONS: T&E regimens are efficacious and safe for nAMD and PCV, can reduce the number of visits, and minimize the overall burden for clinicians and patients.

    Matched MeSH terms: Retina
  7. Razali N, Agarwal R, Agarwal P, Tripathy M, Kapitonova MY, Kutty MK, et al.
    Exp Eye Res, 2016 Feb;143:9-16.
    PMID: 26424219 DOI: 10.1016/j.exer.2015.09.014
    Steroid-induced hypertension and glaucoma is associated with increased extracellular meshwork (ECM) deposition in trabecular meshwork (TM). Previous studies have shown that single drop application of trans-resveratrol lowers IOP in steroid-induced ocular hypertensive (SIOH) rats. This IOP lowering is attributed to activation of adenosine A1 receptors, which may lead to increased matrix metalloproteinase (MMP)-2 activity. This study evaluated the effect of repeated topical application of trans-resveratrol for 21 days in SIOH animals on IOP, changes in MMP-2 level in aqueous humor, trabecular meshwork and retinal morphology and retinal redox status. We observed that treatment with trans-resveratrol results in significant and sustained IOP reduction in SIOH rats. This IOP reduction is associated with significantly higher aqueous humor total MMP-2 level; significantly reduced TM thickness and increased number of TM cells. Treatment with trans-resveratrol also significantly increased ganglion cell layer (GCL) thickness, the linear cell density in the GCL and inner retina thickness; and significantly reduced retinal oxidative stress compared to the SIOH vehicle-treated group. In conclusion, repeated dose topical application of trans-resveratrol produces sustained IOP lowering effect, which is associated with increased level of aqueous humor MMP-2, normalization of TM and retinal morphology and restoration of retinal redox status.
    Matched MeSH terms: Retina/pathology*; Retinal Ganglion Cells/drug effects
  8. Kim Y, Griffin JM, Nor MNM, Zhang J, Freestone PS, Danesh-Meyer HV, et al.
    Neurotherapeutics, 2017 Oct;14(4):1148-1165.
    PMID: 28560708 DOI: 10.1007/s13311-017-0536-9
    The cis benzopyran compound tonabersat (SB-220453) has previously been reported to inhibit connexin26 expression in the brain by attenuating the p38-mitogen-activated protein kinase pathway. We show here that tonabersat directly inhibits connexin43 hemichannel opening. Connexin43 hemichannels have been called "pathological pores" based upon their role in secondary lesion spread, edema, inflammation, and neuronal loss following central nervous system injuries, as well as in chronic inflammatory disease. Both connexin43 hemichannels and pannexin channels released adenosine triphosphate (ATP) during ischemia in an in vitro ischemia model, but only connexin43 hemichannels contributed to ATP release during reperfusion. Tonabersat inhibited connexin43 hemichannel-mediated ATP release during both ischemia and reperfusion phases, with direct channel block confirmed using electrophysiology. Tonabersat also reduced connexin43 gap junction coupling in vitro, but only at higher concentrations, with junctional plaques internalized and degraded via the lysosomal pathway. Systemic delivery of tonabersat in a rat bright-light retinal damage model (a model for dry age-related macular degeneration) resulted in significantly improved functional outcomes assessed using electroretinography. Tonabersat also prevented thinning of the retina, especially the outer nuclear layer and choroid, assessed using optical coherence tomography. We conclude that tonabersat, already given orally to over 1000 humans in clinical trials (as a potential treatment for, and prophylactic treatment of, migraine because it was thought to inhibit cortical spreading depression), is a connexin hemichannel inhibitor and may have the potential to be a novel treatment of central nervous system injury and chronic neuroinflammatory disease.
    Matched MeSH terms: Retina/drug effects; Retina/pathology; Retina/physiopathology
  9. Mohd Lazaldin MA, Iezhitsa I, Agarwal R, Bakar NS, Agarwal P, Mohd Ismail N
    Int J Neurosci, 2018 Oct;128(10):952-965.
    PMID: 29488424 DOI: 10.1080/00207454.2018.1446953
    PURPOSE: Amyloid beta (Aβ) is known to contribute to the pathophysiology of retinal neurodegenerative diseases such as glaucoma. Effects of intravitreal Aβ(1-42) on retinal and optic nerve morphology in animal models have widely been studied but not those of Aβ(1-40). Hence, we evaluated the time- and dose-related effects of intravitreal Aβ(1-40) on retinal and optic nerve morphology. Since oxidative stress and brain derived neurotrophic factor (BDNF) are associated with Aβ-induced neuronal damage, we also studied dose and time-related effects of Aβ(1-40) on retinal oxidative stress and BDNF levels.

    MATERIALS AND METHODS: Five groups of rats were intravitreally administered with vehicle or Aβ(1-40) in doses of 1.0, 2.5, 5 and 10 nmol. Animals were sacrificed and eyes were enucleated at weeks 1, 2 and 4 post-injection. The retinae were subjected to morphometric analysis and TUNEL staining. Optic nerve sections were stained with toluidine blue and were graded for neurodegenerative effects. The estimation of BDNF and markers of oxidative stress in retina were done using ELISA technique.

    RESULTS AND CONCLUSIONS: It was observed that intravitreal Aβ(1-40) causes significant retinal and optic nerve damage up to day 14 post-injection and there was increasing damage with increase in dose. However, on day 30 post-injection both the retinal and optic nerve morphology showed a trend towards normalization. The observations made for retinal cell apoptosis, retinal glutathione, superoxide dismutase activity and BDNF were in accordance with those of morphological changes with deterioration till day 14 and recovery by day 30 post-injection. The findings of this study may provide a guide for selection of appropriate experimental conditions for future studies.

    Matched MeSH terms: Retina
  10. Ooi YL, Khang TF, Naidu M, Fong KC
    Eye (Lond), 2013 Mar;27(3):425-31.
    PMID: 23196646 DOI: 10.1038/eye.2012.260
    To compare the potential retinal toxicity of two commercially Brilliant blue G dyes (Brilliant Peel and Ocublue Plus) and Indocyanine green (ICG) at usual clinical concentration.
    Matched MeSH terms: Retina/drug effects*; Retina/pathology; Retinal Ganglion Cells/drug effects; Retinal Ganglion Cells/pathology; Vitreoretinal Surgery
  11. Kandiah R, Annuar Z, Sukumaran K
    Family Physician, 1989;1:60-63.
    Matched MeSH terms: Retina
  12. Tey MS, Govindasamy G, Vendargon FM
    J Ophthalmic Inflamm Infect, 2020 Nov 16;10(1):31.
    PMID: 33191467 DOI: 10.1186/s12348-020-00224-0
    BACKGROUND: Cat scratch disease (CSD) is a systemic illness caused by the gram-negative bacillus, Bartonella henselea, which can occasionally involve the ocular structures. The objective of this study is to evaluate the various clinical presentations of ocular bartonellosis at our institution. A retrospective review of the clinical records of 13 patients (23 eyes) with ocular manifestations of Bartonella infections over a 3-year period between January 2016 to December 2018 was undertaken at our institution.

    RESULTS: The diagnosis was made based on clinical findings and in addition, with the support of the evidence of Bartonella hensalae IgG and/or IgM. Small retinal white lesions were the most common ocular findings in this series of patients (82.6% of eyes, 76.9% of patients). Neuroretinitis was the second most common finding (47.8% of eyes, 69.2% of patients), followed by exudative retinal detachment involving the macula (34.8% of eyes, 53.8% of patients) and Parinaud's oculoglandular syndrome (17.4% of eyes, 23.1% of patients). Other findings like isolated optic disc oedema without macular star (8.7% of eyes, 15.4% of patients) and vitritis (4.3% of eyes, 7.7% of patients) were also observed. Ten patients (76.9%) had bilateral ocular involvement. Most of the patients were young, immunocompetent and had systemic symptoms like fever prior to their ocular symptoms. The visual acuity (VA) at initial presentation ranged from 6/6 to hand movement (mean, 6/20), and at final visit 6/6 to 6/60, (mean, 6/9). 91.7% of patients were treated with antibiotics. Only 2 patients received oral corticosteroids together with antibiotics due to very poor vision on presentation. The visual prognosis of ocular bartonellosis is generally good with 16 (88.9%) of 23 eyes having VA of 6/12 or better at final follow-up visit.

    CONCLUSION: Small foci of retinal white lesions were the most common manifestation of ocular bartonellosis in this series, followed by neuroretinitis, though an array of other ocular findings may also occur. Therefore, we should consider bartonella infection as a possible differential diagnosis in those patients.

    Matched MeSH terms: Retina; Retinal Detachment
  13. Nor Arfuzir NN, Agarwal R, Iezhitsa I, Agarwal P, Sidek S, Ismail NM
    Neural Regen Res, 2018 Nov;13(11):2014-2021.
    PMID: 30233077 DOI: 10.4103/1673-5374.239450
    Endothelin-1 (ET-1), a potent vasoconstrictor, is involved in retinal vascular dysregulation and oxidative stress in glaucomatous eyes. Taurine (TAU), a naturally occurring free amino acid, is known for its neuroprotective and antioxidant properties. Hence, we evaluated its neuroprotective properties against ET-1 induced retinal and optic nerve damage. ET-1 was administered intravitreally to Sprague-Dawley rats and TAU was injected as pre-, co- or post-treatment. Animals were euthanized seven days post TAU injection. Retinae and optic nerve were examined for morphology, and were also processed for caspase-3 immunostaining. Retinal redox status was estimated by measuring retinal superoxide dismutase, catalase, glutathione, and malondialdehyde levels using enzyme-linked immuosorbent assay. Histopathological examination showed significantly improved retinal and optic nerve morphology in TAU-treated groups. Morphometric examination showed that TAU pre-treatment provided marked protection against ET-1 induced damage to retina and optic nerve. In accordance with the morphological observations, immunostaining for caspase showed a significantly lesser number of apoptotic retinal cells in the TAU pre-treatment group. The retinal oxidative stress was reduced in all TAU-treated groups, and particularly in the pre-treatment group. The findings suggest that treatment with TAU, particularly pre-treatment, prevents apoptosis of retinal cells induced by ET-1 and hence prevents the changes in the morphology of retina and optic nerve. The protective effect of TAU against ET-1 induced retinal and optic nerve damage is associated with reduced retinal oxidative stress.
    Matched MeSH terms: Retina
  14. Mat Nor N, Guo CX, Rupenthal ID, Chen YS, Green CR, Acosta ML
    Invest Ophthalmol Vis Sci, 2018 07 02;59(8):3682-3693.
    PMID: 30029255 DOI: 10.1167/iovs.17-22829
    Purpose: To evaluate the long-term effect on inflammation and inflammasome activation of intravitreally delivered connexin43 mimetic peptide (Cx43MP) in saline or incorporated within nanoparticles (NPs) for the treatment of the light-damaged rat eye.

    Methods: Light-induced damage to the retina was created by exposure of adult albino Sprague-Dawley rats to intense light for 24 hours. A single dose of Cx43MP, Cx43MP-NPs, or saline was injected intravitreally at 2 hours after onset of light damage. Fluorescein isothiocyanate (FITC)-labelled Cx43MP-NPs were intravitreally injected to confirm delivery into the retina. Electroretinogram (ERG) recordings were performed at 24 hours, 1 week, and 2 weeks post cessation of light damage. The retinal and choroidal layers were analyzed in vivo using optical coherence tomography (OCT) and immunohistochemistry was performed on harvested tissues using glial fibrillary acidic protein (GFAP), leukocyte common antigen (CD45), and Cx43 antibodies.

    Results: FITC was visualized 30 minutes after injection in the ganglion cell layer and in the choroid. Cx43MP and Cx43MP-NP treatments improved a-wave and b-wave function of the ERG compared with saline-injected eyes at 1 week and 2 weeks post treatment, and prevented photoreceptor loss by 2 weeks post treatment. Inflammation was also reduced and this was in parallel with downregulation of Cx43 expression.

    Conclusions: The slow release of Cx43MP incorporated into NPs is more effective at treating retinal injury than a single dose of native Cx43MP in solution by reducing inflammation and maintaining both retinal structure and function. This NP preparation has clinical relevance as it reduces possible ocular complications associated with repeated intravitreal injections.

    Matched MeSH terms: Retina/metabolism; Retina/pathology*; Retinal Diseases/drug therapy*; Retinal Diseases/metabolism; Retinal Diseases/pathology
  15. Bastion ML, Qader AM
    BMJ Case Rep, 2011;2011.
    PMID: 22696753 DOI: 10.1136/bcr.02.2011.3889
    The authors would like to present an unusual case of unilateral retinal detachment, phacodonesis, dense cataract and ocular coloboma in a 7-year-old Indian Muslim boy with Noonan syndrome. He underwent lensectomy, 23G pars planar vitrectomy, air fluid exchange, endolaser and silicone oil injection which successfully reattached the retina. His best corrected visual acuity was 6/24 6 months after the surgery and oil removal.
    Matched MeSH terms: Retina/abnormalities*; Retina/surgery; Retinal Detachment/etiology; Retinal Detachment/surgery*
  16. Tengku Kamalden, T.A., Nurliza, K., Haris, A.R.
    JUMMEC, 2008;11(1):30-32.
    MyJurnal
    The presenting signs of acute leukaemia occur as a result of bone marrow failure and organ infiltration. Increased bleeding tendencies are seen on the skin, gums and mucosal lining. Bleeding in the posterior segment of the eye, namely the retina and vitreous, may occur, but do not usually cause any visual disturbances. This case demonstrates visual loss as a result of premacular subhyaloid haemorrhage in acute leukaemia.
    Matched MeSH terms: Retina; Retinal Hemorrhage
  17. Matthew TJH, Tharakan J, Tai E, Hussein A
    Cureus, 2019 Apr 27;11(4):e4553.
    PMID: 31275777 DOI: 10.7759/cureus.4553
    Objective Epilepsy is a debilitating disease. Visual function changes have been reported and may be attributed to the epileptic changes or as a result of medication side effect. Sodium valproate and carbamazepine are both first line anti-epileptic medications used in Malaysian health care. Sodium valproate inhibits glutamate and γ-aminobutyric acid (GABA) transaminase while carbamazepine acts on the sodium channel - both are an important part of the retina. This study aimed to compare the visual functions of epilepsy patients on carbamazepine or sodium valproate monotherapy. Design A cross-sectional study was conducted at a tertiary hospital between June 2016 and November 2018. Methods Patients with idiopathic epilepsy that fulfill the inclusion and exclusion criteria were recruited from the neurology clinic. They were divided into two groups and underwent complete eye examinations. Visual functions such as color vision testing, contrast sensitivity, visual field and retinal nerve fiber layer measurement were subsequently performed. Statistical analysis was done using Statistical Package for the Social Science, version 24 (SPSS Inc, Chicago, IL, USA). Results A total of 100 patients (sodium valproate: 50 patients; carbamazepine: 50 patients) were recruited for the study. There were no statistically significant changes in anatomical or visual function between the sodium valproate and carbamazepine group. However, patients from both groups displayed color vision defect in the blue and green axes. Changes in color vision could indicate early retina toxicity secondary to the medication. Although there were no visual field changes, patients recorded a slight reduction of mean deviation. Changes of mean deviation could be attributed to the side effect of medication or the disease process. Conclusions Epileptic patients taking sodium valproate or carbamazepine did not demonstrate statistically significant change in visual function.
    Matched MeSH terms: Retina
  18. Mohd Khalid MK, Yakob Y, Md Yasin R, Wee Teik K, Siew CG, Rahmat J, et al.
    Mol Vis, 2015;21:1185-90.
    PMID: 26539030
    The availability of molecular genetic testing for retinoblastoma (RB) in Malaysia has enabled patients with a heritable predisposition to the disease to be identified, which thus improves the clinical management of these patients and their families. In this paper, we presented our strategy for performing molecular genetic testing of the RB1 gene and the findings from our first 2 years of starting this service.
    Matched MeSH terms: Retina/metabolism; Retina/pathology; Retinal Neoplasms/ethnology; Retinal Neoplasms/genetics*; Retinal Neoplasms/pathology
  19. Ling KP, Wu AL, Lai CC, Wu WC
    Taiwan J Ophthalmol, 2019 1 15;8(4):249-252.
    PMID: 30637197 DOI: 10.4103/tjo.tjo_100_18
    We report a case with multiple recurrences of retinal detachment (RD) with severe proliferative vitreoretinopathy in a 12-year-old child who underwent vitrectomy using a heavy liquid perfluoro-n-octane (PFO) as a short-term postoperative tamponade. He had an encircling band and three vitrectomies with gas, followed by silicone oil tamponade for retinal redetachment prior to the use of PFO as a short-term tamponade. Short-term PFO tamponade was used in which inferior retinal reattachment was considered to be difficult with conventional gas or silicone oil tamponade, especially in regard to proper postoperative posturing. The PFO was left in the eye for 1 week, and the retina remained reattached after removal of the PFO which was replaced with silicone oil tamponade in this case. PFO appears feasible and tolerable as a short-term postoperative tamponade in the management of complex pediatric RD.
    Matched MeSH terms: Retina; Retinal Detachment
  20. Paraoan L, Sharif U, Carlsson E, Supharattanasitthi W, Mahmud NM, Kamalden TA, et al.
    Prog Retin Eye Res, 2020 11;79:100859.
    PMID: 32278708 DOI: 10.1016/j.preteyeres.2020.100859
    Secretory proteostasis integrates protein synthesis, processing, folding and trafficking pathways that are essential for efficient cellular secretion. For the retinal pigment epithelium (RPE), secretory proteostasis is of vital importance for the maintenance of the structural and functional integrity of apical (photoreceptors) and basal (Bruch's membrane/choroidal blood supply) sides of the environment it resides in. This integrity is achieved through functions governed by RPE secreted proteins, which include extracellular matrix modelling/remodelling, angiogenesis and immune response modulation. Impaired RPE secretory proteostasis affects not only the extracellular environment, but leads to intracellular protein aggregation and ER-stress with subsequent cell death. Ample recent evidence implicates dysregulated proteostasis as a key factor in the development of age-related macular degeneration (AMD), the leading cause of blindness in the developed world, and research aiming to characterise the roles of various proteins implicated in AMD-associated dysregulated proteostasis unveiled unexpected facets of the mechanisms involved in degenerative pathogenesis. This review analyses cellular processes unveiled by the study of the top 200 transcripts most abundantly expressed by the RPE/choroid in the light of the specialised secretory nature of the RPE. Functional roles of these proteins and the mechanisms of their impaired secretion, due to age and genetic-related causes, are analysed in relation to AMD development. Understanding the importance of RPE secretory proteostasis in relation to maintaining retinal health and how it becomes impaired in disease is of paramount importance for the development and assessment of future therapeutic advancements involving gene and cell therapies.
    Matched MeSH terms: Retina/metabolism*; Retina/pathology; Retinal Pigment Epithelium/metabolism*; Retinal Pigment Epithelium/pathology
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