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Endothelial corneal dystrophy

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https://www.readbyqxmd.com/read/28934248/vps35-deficiency-impairs-slc4a11-trafficking-and-promotes-corneal-dystrophy
#1
Wei Liu, Fu-Lei Tang, Sen Lin, Kai Zhao, Lin Mei, Jian Ye, Wen-Cheng Xiong
Vps35 (vacuolar protein sorting 35) is a major component of retromer that selectively promotes endosome-to-Golgi retrieval of transmembrane proteins. Dysfunction of retromer is a risk factor for the pathogenesis of Parkinson's disease (PD) and Alzheimer's disease (AD). However, Vps35/retromer's function in the eye or the contribution of Vps35-deficiency to eye degenerative disorders remains to be explored. Here we provide evidence for a critical role of Vps35 in mouse corneal dystrophy. Vps35 is expressed in mouse and human cornea...
2017: PloS One
https://www.readbyqxmd.com/read/28886202/fuchs-endothelial-corneal-dystrophy-and-rna-foci-in-patients-with-myotonic-dystrophy
#2
V Vinod Mootha, Brock Hansen, Ziye Rong, Pradeep P Mammen, Zhengyang Zhou, Chao Xing, Xin Gong
Purpose: The most common cause of Fuchs' endothelial corneal dystrophy (FECD) is an intronic CTG repeat expansion in TCF4. Expanded CUG repeat RNA colocalize with splicing factor, muscleblind-like 1 (MBNL1), in nuclear foci in endothelium as a molecular hallmark. Myotonic dystrophy type 1 (DM1) is a neuromuscular disorder caused by a CTG repeat expansion in the 3'-untranslated region (UTR) of DMPK. In this study, we examine for RNA-MBNL1 foci in endothelial cells of FECD subjects with DM1, test the hypothesis that DM1 patients are at risk for FECD, and determine prevalence of TCF4 and DMPK expansions in a FECD cohort...
September 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28835226/intraocular-lens-power-calculation-using-standard-formulas-and-ray-tracing-after-dmek-in-patients-with-fuchs-endothelial-dystrophy
#3
Maged Alnawaiseh, Lars Zumhagen, André Rosentreter, Nicole Eter
BACKGROUND: The study presented here aims to optimize the accuracy of intraocular lens (IOL) power calculations in patients after DMEK by evaluation of the impact of the altered anterior/posterior corneal curvature relationship. METHODS: Scheimpflug-based Oculus Pentacam imaging was performed after DMEK surgery for Fuchs endothelial dystrophy. The IOL power was calculated for all patients by ray tracing, aiming for postoperative emmetropia. We also performed the IOL calculation using four third-generation formulas (SRK-T, Hoffer-Q, Holladay-1 and Haigis)...
August 23, 2017: BMC Ophthalmology
https://www.readbyqxmd.com/read/28832669/tgc-repeat-expansion-in-the-tcf4-gene-increases-the-risk-of-fuchs-endothelial-corneal-dystrophy-in-australian-cases
#4
Abraham Kuot, Alex W Hewitt, Grant R Snibson, Emmanuelle Souzeau, Richard Mills, Jamie E Craig, Kathryn P Burdon, Shiwani Sharma
Fuchs' endothelial corneal dystrophy (FECD) is a progressive, vision impairing disease. Common single nucleotide polymorphisms (SNPs) and a trinucleotide repeat polymorphism, thymine-guanine-cytosine (TGC), in the TCF4 gene have been associated with the risk of FECD in some populations. We previously reported association of SNPs in TCF4 with FECD risk in the Australian population. The aim of this study was to determine whether TGC repeat polymorphism in TCF4 is associated with FECD in the Australian population...
2017: PloS One
https://www.readbyqxmd.com/read/28820792/corneal-endothelial-dystrophy-associated-with-myotonic-dystrophy-a-report-of-2-cases
#5
Julia Furtado Heringer, Ruth Miyuki Santo, Leonardo José Barbosa, Amaryllis Avakian, Pedro Carlos Carricondo
No abstract text is available yet for this article.
October 2017: Cornea
https://www.readbyqxmd.com/read/28801092/anterior-segment-optical-coherence-tomography-for-the-diagnosis-of-corneal-dystrophies-according-to-the-ic3d-classification
#6
REVIEW
Sebastian Siebelmann, Paula Scholz, Simon Sonnenschein, Björn Bachmann, Mario Matthaei, Claus Cursiefen, Ludwig M Heindl
Corneal dystrophies are categorized according to the International Committee for Classification of Corneal Dystrophies (IC3D) classification, and their treatment depends on the affected structures and layer of the cornea. Therefore, estimating the depth and extent of the morphological changes due to the specific dystrophy is crucial when deciding between different treatment options. Besides superficial laser treatments and penetrating keratoplasty, minimal invasive lamellar keratoplasties such as Descemet membrane endothelial keratoplasty), deep anterior lamellar keratoplasty, or Descemet stripping automated keratoplasty have become increasingly popular to exchange the specific opaque layers in dystrophic eyes...
August 8, 2017: Survey of Ophthalmology
https://www.readbyqxmd.com/read/28782089/the-role-of-lipids-in-corneal-diseases-and-dystrophies-a-systematic-review
#7
REVIEW
Tyler G Rowsey, Dimitrios Karamichos
Corneal diseases are an extensive cause of blindness worldwide and continue to persist as a challenging public health concern. Recently, various lipid-based therapies have been advocated for the modulation of corneal diseases; however, the number of studies is still very limited. Here we focus on developments and challenges on lipid-based therapies for dry eye disease, diabetic neuropathy, and Fuchs' endothelial corneal dystrophy. All three diseases are highly prevalent conditions and involve corneal stress and inflammation...
December 2017: Clinical and Translational Medicine
https://www.readbyqxmd.com/read/28754918/activation-of-tgf-%C3%AE-signaling-induces-cell-death-via-the-unfolded-protein-response-in-fuchs-endothelial-corneal-dystrophy
#8
Naoki Okumura, Keisuke Hashimoto, Miu Kitahara, Hirokazu Okuda, Emi Ueda, Kyoko Watanabe, Makiko Nakahara, Takahiko Sato, Shigeru Kinoshita, Theofilos Tourtas, Ursula Schlötzer-Schrehardt, Friedrich Kruse, Noriko Koizumi
Fuchs endothelial corneal dystrophy (FECD) is a slowly progressive bilateral disease of corneal endothelium in which accumulation of extracellular matrix (ECM) and loss of corneal endothelial cells (CECs) are phenotypic features. The corneal endothelium maintains corneal transparency by regulating water hydration; consequently, corneal endothelial dysfunction causes serious vision loss. The only therapy for corneal haziness due to corneal endothelial diseases, including FECD, is corneal transplantation using donor corneas, and no pharmaceutical treatment is available...
July 28, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28751984/corneal-backscatters-as-an-objective-index-for-assessing-fuchs-endothelial-corneal-dystrophy-a-pilot-study
#9
Hsueh-Yen Chu, Ching-Hsi Hsiao, Phil Yeong-Fong Chen, David Hui-Kang Ma, Chee-Jen Chang, Hsin-Yuan Tan
PURPOSE: To provide an objective, quantitative approach for monitoring Fuchs' endothelial corneal dystrophy (FECD), with Scheimpflug imaging. DESIGN: This is a retrospective case-control pilot study. METHODS: The study group consisted of 53 eyes in 27 patients diagnosed with FECD, with normal subjects paired as control. Main outcome measures were corneal thickness, morphological patterns on densitograms, and indices of corneal density including the average area density (mean AD) and the average ratio of Descemet's membrane density versus area density (DM/AD) in Pentacam Scheimpflug images...
2017: Journal of Ophthalmology
https://www.readbyqxmd.com/read/28751712/activation-of-mitophagy-leads-to-decline-in-mfn2-and-loss-of-mitochondrial-mass-in-fuchs-endothelial-corneal-dystrophy
#10
Anne-Sophie Benischke, Shivakumar Vasanth, Takashi Miyai, Kishore Reddy Katikireddy, Tomas White, Yuming Chen, Adna Halilovic, Marianne Price, Francis Price, Paloma B Liton, Ula V Jurkunas
Human corneal endothelial cells (HCEnCs) are terminally differentiated cells that have limited regenerative potential. The large numbers of mitochondria in HCEnCs are critical for pump and barrier function required for corneal hydration and transparency. Fuchs Endothelial Corneal Dystrophy (FECD) is a highly prevalent late-onset oxidative stress disorder characterized by progressive loss of HCEnCs. We previously reported increased mitochondrial fragmentation and reduced ATP and mtDNA copy number in FECD. Herein, carbonyl cyanide m-chlorophenyl hydrazone (CCCP)-induced mitochondrial depolarization decreased mitochondrial mass and Mfn2 levels, which were rescued with mitophagy blocker, bafilomycin, in FECD...
July 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28749896/elimination-of-anterior-corneal-steepening-with-descemet-membrane-endothelial-keratoplasty-in-a-patient-with-fuchs-dystrophy-and-keratoconus-implications-for-iol-calculation
#11
Reena Gupta, Ruta Kinderyte, Deborah S Jacobs, Ula V Jurkunas
PURPOSE: To report a case of coexistent Fuchs endothelial corneal dystrophy (FECD) and keratoconus (KCN) in which there was normalization of corneal topography after Descemet membrane endothelial keratoplasty (DMEK). METHODS: Retrospective medical record review. RESULTS: Preoperative findings revealed a best-corrected visual acuity of 20/40 with -1.00 - 2.50 × 147, topographic maximum keratometry of 50.8 D with inferior steeping, and confluent guttae in the left eye...
October 2017: Cornea
https://www.readbyqxmd.com/read/28742617/simple-single-pass-technique-for-ultrathin-descemet-stripping-automated-endothelial-keratoplasty-a-pilot-study
#12
Maria E S Dimitry, Adam D Lewis, Fani Zacharaki, Manju Chandran, Deana Robson, Geeta Menon, Thomas R G Poole
PURPOSE: To assess intraoperative and postoperative graft thickness (GT) after donor deturgescence for ultrathin Descemet stripping automated endothelial keratoplasty and to evaluate visual outcomes, endothelial cell density, and patient satisfaction at 1 year. METHODS: Prospective interventional case series of patients with Fuchs endothelial dystrophy, Fuchs endothelial dystrophy and cataract, and pseudophakic bullous keratopathy (n = 12 grafts). The donor cornea was allowed to thin out by simple evaporation on an artificial anterior chamber, to the required precut thickness, before a single microkeratome pass...
October 2017: Cornea
https://www.readbyqxmd.com/read/28738416/conditionally-immortal-slc4a11-mouse-corneal-endothelial-cell-line-recapitulates-disrupted-glutaminolysis-seen-in-slc4a11-mouse-model
#13
Wenlin Zhang, Diego G Ogando, Edward T Kim, Moon-Jung Choi, Hongde Li, Jason M Tenessen, Joseph A Bonanno
Purpose: To establish conditionally immortal mouse corneal endothelial cell lines with genetically matched Slc4a11+/+ and Slc4a11-/- mice as a model for investigating pathology and therapies for SLC4A11 associated congenital hereditary endothelial dystrophy (CHED) and Fuchs' endothelial corneal dystrophy. Methods: We intercrossed H-2Kb-tsA58 mice (Immortomouse) expressing an IFN-γ dependent and temperature-sensitive mutant of the SV40 large T antigen (tsTAg) with Slc4a11+/+ and Slc4a11-/- C57BL/6 mice...
July 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28731879/peripheral-endothelial-cell-count-is-a-predictor-of-disease-severity-in-advanced-fuchs-endothelial-corneal-dystrophy
#14
Zeba A Syed, Jennifer A Tran, Ula V Jurkunas
PURPOSE: In advanced Fuchs endothelial corneal dystrophy (FECD), central endothelial changes do not correlate with disease severity. The peripheral endothelial cell count (ECC) has not been studied as a marker of FECD severity. The goal of this study was to determine the relationship between the peripheral ECC and known clinical markers of FECD in advanced cases. METHODS: Patients with FECD examined between January 1, 2013, and September 1, 2016, by 1 cornea specialist were identified...
October 2017: Cornea
https://www.readbyqxmd.com/read/28727885/sustained-activation-of-the-unfolded-protein-response-induces-cell-death-in-fuchs-endothelial-corneal-dystrophy
#15
Naoki Okumura, Miu Kitahara, Hirokazu Okuda, Keisuke Hashimoto, Emi Ueda, Makiko Nakahara, Shigeru Kinoshita, Robert D Young, Andrew J Quantock, Theofilos Tourtas, Ursula Schlötzer-Schrehardt, Friedrich Kruse, Noriko Koizumi
Purpose: The unfolded protein response (UPR) is believed to play a role in the pathogenesis of Fuchs' endothelial corneal dystrophy (FECD). The purpose of this study was to investigate whether unfolded proteins accumulate in the corneal endothelium in FECD and if they are involved in triggering cell death. Methods: Descemet's membranes with corneal endothelial cells (CECs) were obtained during keratoplasty, and expression of aggresomes, type 1 collagen, fibronectin, and agrin was evaluated...
July 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28726551/extracellular-matrix-and-integrin-expression-profiles-in-fuchs-endothelial-corneal-dystrophy-cells-and-tissue-model
#16
Benjamin Goyer, Mathieu Thériault, Sébastien P Gendron, Isabelle Brunette, Patrick J Rochette, Stéphanie Proulx
Primary corneal endothelial cell cultures and 3D engineered tissue models were used to study the aberrant deposition of extracellular matrix (ECM) in a vision impairing pathology known as Fuchs endothelial corneal dystrophy (FECD). Corneal endothelial cells (CEC) were isolated from excised Descemet membranes of patients with end-stage FECD. CEC isolated from healthy corneas served as controls. Microarray gene profiling was performed on post-confluent cultures of healthy and FECD cells. Protein expression analyses were conducted on tissue models that were engineered by seeding an endothelium on previously devitalized human stromal carriers...
July 20, 2017: Tissue Engineering. Part A
https://www.readbyqxmd.com/read/28726355/combined-cataract-dsek-dmek-changing-expectations
#17
Francis W Price, Marianne O Price
Endothelial keratoplasty (EK) has revolutionized corneal transplant surgery by providing rapid visual recovery and improved visual outcomes. In parts of the world with Fuchs endothelial dystrophy, many patients may present with both cataracts and corneal degeneration requiring surgery. Other forms of endothelial decompensation may also present with cataracts. The staging or combination of transplant surgery and cataract surgery depend on both the abilities of the surgeon and the surgical techniques being considered...
July 2017: Asia-Pacific Journal of Ophthalmology
https://www.readbyqxmd.com/read/28697678/ek-dlek-dsek-dmek-new-frontier-in-cornea-surgery
#18
Marianne O Price, Pankaj Gupta, Jonathan Lass, Francis W Price
Endothelial keratoplasty (EK) has revolutionized treatment of corneal endothelial dysfunction. Compared with penetrating keratoplasty (PK), EK provides faster and more reliable visual rehabilitation while maintaining the eye's structural integrity. The number of EK procedures is growing annually and surpassed PK in the United States in 2012. The most widely used iteration, Descemet stripping endothelial keratoplasty (DSEK), implants healthy donor endothelium, Descemet membrane, and posterior stroma. Descemet membrane endothelial keratoplasty (DMEK) eliminates the donor stromal layer...
September 15, 2017: Annual Review of Vision Science
https://www.readbyqxmd.com/read/28683480/-penetrating-limbokeratoplasty-for-gelatinous-corneal-dystrophy
#19
Stefan J Lang, Daniel Böhringer, Thomas Reinhard
Background Gelatinous corneal dystrophy is a very rare disease in Europe and North America. Patients suffer from pain, photophobia and loss of vision, usually before the age of 20. Transplantation of limbal stem cells was proposed due to the high rate of recurrence after conventional penetrating keratoplasty. We present the results of penetrating limbokeratoplasty, a combined transplantation of cornea and limbal stem cells, in patients with gelatinous corneal dystrophy. Methods We present a series of 7 consecutive eyes with gelatinous corneal dystrophy, which were treated with penetrating limbokeratoplasty-4 eyes of 3 patients had already undergone multiple conventional corneal transplantations...
July 6, 2017: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/28654985/transcriptomic-profiling-of-posterior-polymorphous-corneal-dystrophy
#20
Doug D Chung, Ricardo F Frausto, Benjamin R Lin, Evelyn M Hanser, Zack Cohen, Anthony J Aldave
Purpose: To investigate the molecular basis of posterior polymorphous corneal dystrophy (PPCD) by examining the PPCD transcriptome and the effect of decreased ZEB1 expression on corneal endothelial cell (CEnC) gene expression. Methods: Next-generation RNA sequencing (RNA-seq) analyses of corneal endothelium from two PPCD-affected individuals (one with PPCD3 and one of unknown genetic cause) compared with two age-matched controls, and primary human CEnC (pHCEnC) transfected with siRNA-mediated ZEB1 knockdown...
June 1, 2017: Investigative Ophthalmology & Visual Science
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