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Joshua Dunaief

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https://read.qxmd.com/read/30864945/impaired-abca1-abcg1-mediated-lipid-efflux-in-the-mouse-retinal-pigment-epithelium-rpe-leads-to-retinal-degeneration
#1
Federica Storti, Katrin Klee, Vyara Todorova, Regula Steiner, Alaa Othman, Saskia van der Velde-Visser, Marijana Samardzija, Isabelle Meneau, Maya Barben, Duygu Karademir, Valda Pauzuolyte, Sanford L Boye, Frank Blaser, Christoph Ullmer, Joshua L Dunaief, Thorsten Hornemann, Lucia Rohrer, Anneke I den Hollander, Arnold von Eckardstein, Jürgen Fingerle, Cyrille Maugeais, Christian Grimm
Age-related macular degeneration (AMD) is a progressive disease of the retinal pigment epithelium (RPE) and the retina leading to loss of central vision. Polymorphisms in genes involved in lipid metabolism, including the ATP-binding cassette transporter A1 ( ABCA1 ), have been associated with AMD risk. However, the significance of retinal lipid handling for AMD pathogenesis remains elusive. Here, we study the contribution of lipid efflux in the RPE by generating a mouse model lacking ABCA1 and its partner ABCG1 specifically in this layer...
March 13, 2019: ELife
https://read.qxmd.com/read/30711487/complement-factor-h-mutation-w1206r-causes-retinal-thrombosis-and-ischemic-retinopathy-in-mice
#2
Delu Song, Yoshiyasu Ueda, Rupak Bhuyan, Imran Mohammed, Takashi Miwa, Damodar Gullipali, Hangsoo Kim, Lin Zhou, Ying Song, Hannah Schultz, Albert Bargoud, Joshua L Dunaief, Wen-Chao Song
Single-nucleotide polymorphisms and rare mutations in factor H (FH; official name, CFH) are associated with age-related macular degeneration and atypical hemolytic uremic syndrome, a form of thrombotic microangiopathy. Mice with the FH W1206R mutation (FHR/R ) share features with human atypical hemolytic uremic syndrome. Herein, we report that FHR/R mice exhibited retinal vascular occlusion and ischemia. Retinal fluorescein angiography demonstrated delayed perfusion and vascular leakage in FHR/R mice. Optical coherence tomography imaging of FHR/R mice showed retinal degeneration, edema, and detachment...
February 1, 2019: American Journal of Pathology
https://read.qxmd.com/read/30665232/selective-ablation-of-megalin-in-the-retinal-pigment-epithelium-results-in-megaophthalmos-macromelanosome-formation-and-severe-retina-degeneration
#3
Tina Storm, Thomas Burgoyne, Joshua L Dunaief, Erik I Christensen, Clare Futter, Rikke Nielsen
Purpose: Mutations in the megalin-encoding gene, LRP2, cause high myopia as seen in patients suffering from Donnai-Barrow/facio-oculo-acoustico-renal syndrome. Megalin is present in both the nonpigmented epithelium of the ciliary body and in the RPE. In this study, we set out to establish an animal model to study the mechanisms underlying the ocular phenotype and to establish if high myopia/megaophthalmos is induced by postnatal megalin-deficiency in the RPE. Methods: Postnatal RPE-specific deletion of megalin was generated by crossing mice bearing a homozygous loxP-flanked Lrp2 allele with transgenic mice expressing the Cre recombinase driven by the BEST1 promotor...
January 2, 2019: Investigative Ophthalmology & Visual Science
https://read.qxmd.com/read/30653965/comparative-localization-of-cystathionine-beta-synthases-and-cystathionine-gamma-lyase-in-canine-non-human-primate-and-human-retina
#4
Alireza Badiei, Raghavi Sudharsan, Evelyn Santana, Joshua L Dunaief, Gustavo D Aguirre
Chronic exposure of the retina to light and high concentrations of polyunsaturated fatty acid in photoreceptor cells make this tissue susceptible to oxidative damage. As retinal degenerative diseases are associated with photoreceptor degeneration, the antioxidant activity of both hydrogen sulfide (H2 S) and glutathione (GSH) may play an important role in ameliorating disease progression. H2 S production is driven by cystathionine-γ-lyase (CSE) and cystathionine-β-synthase (CBS), the key enzymes that also drive transsulfuration pathway (TSP) necessary for GSH production...
January 14, 2019: Experimental Eye Research
https://read.qxmd.com/read/30476517/five-year-follow-up-of-nonfibrotic-scars-in-the-comparison-of-age-related-macular-degeneration-treatments-trials
#5
Ebenezer Daniel, Gui-Shuang Ying, Benjamin J Kim, Cynthia A Toth, Frederick Ferris, Daniel F Martin, Juan E Grunwald, Glenn J Jaffe, Joshua L Dunaief, Wei Pan, Maureen G Maguire
PURPOSE: To describe changes in visual acuity (VA) and macular morphologic features at 5 years in eyes with nonfibrotic scar (NFS) identified at 1 year in the Comparison of Age-Related Macular Degeneration Treatments Trials (CATT). DESIGN: Prospective cohort study within a randomized clinical trial. PARTICIPANTS: Participants in CATT. METHODS: Participants assigned to ranibizumab or bevacizumab and to 1 of 3 dosing regimens were released from the clinical trial protocol after 2 years and recalled at 5 years...
November 23, 2018: Ophthalmology
https://read.qxmd.com/read/30360383/potential-treatment-of-retinal-diseases-with-iron-chelators
#6
REVIEW
Wanting Shu, Joshua L Dunaief
Iron is essential for life, while excess iron can be toxic. Iron generates hydroxyl radical, which is the most reactive free radical, causing oxidative stress. Since iron is absorbed through the diet but not excreted from the body, it accumulates with age in tissues, including the retina, consequently leading to age-related toxicity. This accumulation is further promoted by inflammation. Hereditary diseases such as aceruloplasminemia, Friedreich's ataxia, pantothenate kinase-associated neurodegeneration, and posterior column ataxia with retinitis pigmentosa involve retinal degeneration associated with iron dysregulation...
October 22, 2018: Pharmaceuticals
https://read.qxmd.com/read/30355956/speckled-hypoautofluorescence-as-a-sign-of-resolved-subretinal-hemorrhage-in-neovascular-age-related-macular-degeneration
#7
S Amal Hussnain, Rosa Dolz-Marco, Joshua L Dunaief, Christine A Curcio, K Bailey Freund
PURPOSE: To describe patterns of hypoautofluorescence in eyes with neovascular age-related macular degeneration occurring after subretinal hemorrhage. METHODS: This was a retrospective descriptive analysis of neovascular age-related macular degeneration eyes presenting with subretinal hemorrhage over the last 5 years that underwent serial multimodal imaging. A review of color fundus photographs, fundus autofluorescence, near-infrared reflectance, and optical coherence tomography was performed at baseline and all available follow-up visits to document the course and evolution of subretinal hemorrhage in these eyes...
October 22, 2018: Retina
https://read.qxmd.com/read/30333155/mechanisms-that-minimize-retinal-impact-of-apolipoprotein-e-absence
#8
Aicha Saadane, Alexey Petrov, Natalia Mast, Nicole El-Darzi, Tung Dao, Ahab Alnemri, Ying Song, Joshua L Dunaief, Irina A Pikuleva
Apolipoprotein E (APOE) is a component of lipid-transporting particles and a recognition ligand for receptors, which bind these particles. The APOE isoform 2 is a risk factor for age-related macular degeneration; nevertheless APOE absence in humans and mice does not significantly affect the retina. We found that retinal cholesterol biosynthesis and the levels of retinal cholesterol were increased in Apoe-/- mice, whereas cholesterol elimination by metabolism was decreased. No focal cholesterol deposits were observed in the Apoe-/- retina...
October 17, 2018: Journal of Lipid Research
https://read.qxmd.com/read/30182051/severe-iron-metabolism-defects-in-mice-with-double-knockout-of-the-multicopper-ferroxidases-hephaestin-and-ceruloplasmin
#9
Brie K Fuqua, Yan Lu, David M Frazer, Deepak Darshan, Sarah J Wilkins, Linda Dunn, Alex V Loguinov, Scott C Kogan, Pavle Matak, Huijun Chen, Joshua L Dunaief, Chris D Vulpe, Gregory J Anderson
Background & Aims: Multicopper ferroxidases (MCFs) facilitate intestinal iron absorption and systemic iron recycling, likely by a mechanism involving the oxidization of Fe2+ from the iron exporter ferroportin 1 for delivery to the circulating Fe3+ carrier transferrin. Hephaestin (HEPH), the only MCF known to be expressed in enterocytes, aids in the basolateral transfer of dietary iron to the blood. Mice lacking HEPH in the whole body ( Heph -/- ) or intestine alone ( Heph int/int ) exhibit defects in dietary iron absorption but still survive and grow...
2018: Cellular and Molecular Gastroenterology and Hepatology
https://read.qxmd.com/read/30025090/retinal-basal-laminar-deposits-in-complement-fh-fp-mouse-model-of-dense-deposit-disease
#10
Delu Song, Imran Mohammed, Rupak Bhuyan, Takashi Miwa, Allison Lesher Williams, Damodar Gullipalli, Sayaka Sato, Ying Song, Joshua L Dunaief, Wen-Chao Song
Purpose: Dense deposit disease (DDD) is caused by dysregulation of the alternative pathway of the complement cascade and characterized by electron-dense deposits in the kidney glomerular basement membrane (GBM) and drusen in Bruch's membrane (BrM). Complement factor H (fH) and factor properdin (fP) regulate complement activation; fH inhibits alternative pathway (AP) activation, whereas fP promotes it. We report pathologic changes in eyes of an fH and fP double-mutant mouse, which we previously showed have dense deposits in the GBM and early mortality from nephropathy...
July 2, 2018: Investigative Ophthalmology & Visual Science
https://read.qxmd.com/read/29686088/iron-promotes-oxidative-cell-death-caused-by-bisretinoids-of-retina
#11
Keiko Ueda, Hye Jin Kim, Jin Zhao, Ying Song, Joshua L Dunaief, Janet R Sparrow
Intracellular Fe plays a key role in redox active energy and electron transfer. We sought to understand how Fe levels impact the retina, given that retinal pigment epithelial (RPE) cells are also challenged by accumulations of vitamin A aldehyde adducts (bisretinoid lipofuscin) that photogenerate reactive oxygen species and photodecompose into damaging aldehyde- and dicarbonyl-bearing species. In mice treated with the Fe chelator deferiprone (DFP), intracellular Fe levels, as reflected in transferrin receptor mRNA expression, were reduced...
May 8, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://read.qxmd.com/read/29659961/multi-copper-ferroxidase-deficient-mice-have-increased-brain-iron-concentrations-and-learning-and-memory-deficits
#12
Jiashuo Zheng, Ruiwei Jiang, Min Chen, Zaitunamu Maimaitiming, Junzhuo Wang, Gregory J Anderson, Chris D Vulpe, Joshua L Dunaief, Huijun Chen
Background: The accumulation of iron occurs in the central nervous system (CNS) in several neurodegenerative diseases. Although multi-copper ferroxidases (MCFs) play an important role in cellular iron metabolism and homeostasis, the mechanism of MCFs in the CNS remains unclear. Objective: The aim was to study the role of MCFs in CNS iron metabolism and homeostasis by using hephaestin/ceruloplasmin (Heph/Cp) double knockout (KO) mice. Methods: Heph/Cp double KO male mice were generated by crossing both single KO mice...
April 1, 2018: Journal of Nutrition
https://read.qxmd.com/read/28887373/optical-coherence-tomography-identifies-outer-retina-thinning-in-frontotemporal-degeneration
#13
Benjamin J Kim, David J Irwin, Delu Song, Ebenezer Daniel, Jennifer D Leveque, Aaishah R Raquib, Wei Pan, Gui-Shuang Ying, Tomas S Aleman, Joshua L Dunaief, Murray Grossman
OBJECTIVE: Whereas Alzheimer disease (AD) is associated with inner retina thinning visualized by spectral-domain optical coherence tomography (SD-OCT), we sought to determine if the retina has a distinguishing biomarker for frontotemporal degeneration (FTD). METHODS: Using a cross-sectional design, we examined retinal structure in 38 consecutively enrolled patients with FTD and 44 controls using a standard SD-OCT protocol. Retinal layers were segmented with the Iowa Reference Algorithm...
October 10, 2017: Neurology
https://read.qxmd.com/read/28846772/conditional-m%C3%A3-ller-cell-ablation-leads-to-retinal-iron-accumulation
#14
Bailey Baumann, Jacob Sterling, Ying Song, Delu Song, Marcus Fruttiger, Mark Gillies, Weiyong Shen, Joshua L Dunaief
Purpose: Retinal iron accumulation is observed in a wide range of retinal degenerative diseases, including AMD. Previous work suggests that Müller glial cells may be important mediators of retinal iron transport, distribution, and regulation. A transgenic model of Müller cell loss recently demonstrated that primary Müller cell ablation leads to blood-retinal barrier leakage and photoreceptor degeneration, and it recapitulates clinical features observed in macular telangiectasia type 2 (MacTel2), a rare human disease that features Müller cell loss...
August 1, 2017: Investigative Ophthalmology & Visual Science
https://read.qxmd.com/read/28458506/intraperitoneal-injection-of-epigallocatechin-3-gallate-protects-against-light-induced-photoreceptor-degeneration-in-the-mouse-retina
#15
Shounan Qi, Chenguang Wang, Delu Song, Ying Song, Joshua L Dunaief
PURPOSE: (-)-epigallocatechin-3-gallate (EGCG), a major catechin component of green tea, is reported to delay or prevent certain forms of cancer, arthritis, cardiovascular disease, and neurodegenerative disorders. In this study, we determined if systemically administered EGCG could protect the retina against light damage (LD) in mice. METHODS: BALB/cJ mice were treated with either EGCG or saline via intraperitoneal (IP) injection, and then placed under constant cool white light-emitting diode (LED) light (10,000 lux) for 5 h...
2017: Molecular Vision
https://read.qxmd.com/read/28442885/complement-c5a-receptor-knockout-has-diminished-light-induced-microglia-macrophage-retinal-migration
#16
COMPARATIVE STUDY
Delu Song, Michael E Sulewski, Chenguang Wang, Jiantao Song, Rupak Bhuyan, Jacob Sterling, Esther Clark, Wen-Chao Song, Joshua L Dunaief
PURPOSE: The complement system is involved in the pathogenesis of age-related macular degeneration (AMD). Because activated microglia are also associated with AMD, we studied the relationship between complement anaphylatoxin receptors and microglial recruitment. METHODS: We assessed the effect of anaphylatoxin C3a receptor (C3aR) and C5a receptor (C5aR) knockout (KO) on light damage-induced migration of microglia/macrophages into the mouse outer retina via immunofluorescence and real-time quantitative PCR...
2017: Molecular Vision
https://read.qxmd.com/read/28057640/murine-systemic-thrombophilia-and-hemolytic-uremic-syndrome-from-a-factor-h-point-mutation
#17
Yoshiyasu Ueda, Imran Mohammed, Delu Song, Damodar Gullipalli, Lin Zhou, Sayaka Sato, Yuan Wang, Shuchi Gupta, Zhongjian Cheng, Hong Wang, Jialing Bao, Yingying Mao, Lawrence Brass, X Long Zheng, Takashi Miwa, Matthew Palmer, Joshua Dunaief, Wen-Chao Song
Complement plays a key role in host defense, but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins, including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic uremic syndrome, a type of thrombotic microangiopathy (TMA) that causes renal failure. We describe here that disruption of FH function on the cell surface can also lead to disseminated complement-dependent macrovascular thrombosis...
March 2, 2017: Blood
https://read.qxmd.com/read/28057442/iron-importers-zip8-and-zip14-are-expressed-in-retina-and-regulated-by-retinal-iron-levels
#18
Jacob Sterling, Samyuktha Guttha, Ying Song, Delu Song, Majda Hadziahmetovic, Joshua L Dunaief
Intracellular retinal iron accumulation has been implicated in the pathogenesis of age-related macular degeneration (AMD), the leading cause of irreversible blindness among individuals over the age of 50. Ceruloplasmin/hephaestin double knockout mice (Cp/Heph DKO) and hepcidin knockout mice (Hepc KO) accumulate retinal iron and model some features of AMD. Two canonical pathways govern cellular iron import - transferrin-bound iron import and non-transferrin bound iron import. In Cp/Heph DKO and Hepc KO iron-loaded retinas, transferrin-bound iron import is downregulated...
February 2017: Experimental Eye Research
https://read.qxmd.com/read/27991585/hephaestin-and-ceruloplasmin-facilitate-iron-metabolism-in-the-mouse-kidney
#19
Bo Jiang, Guohao Liu, Jiashuo Zheng, Mengxia Chen, Zaitunamu Maimaitiming, Min Chen, Shunli Liu, Ruiwei Jiang, Brie K Fuqua, Joshua L Dunaief, Chris D Vulpe, Gregory J Anderson, Hongwei Wang, Huijun Chen
Multicopper ferroxidases (MCFs) play an important role in cellular iron homeostasis. However, the role of MCFs in renal metabolism remains unclear. We used Hephaestin (Heph) and Ceruloplasmin (Cp) single or double (Heph/Cp) knockout (KO) mice to study the roles of MCFs in the kidney. Renal iron levels and the expression of iron metabolism genes were examined. The non-heme iron content both in the renal cortex and medulla of Heph/Cp KO mice was significantly increased. Perls' Prussian blue staining showed iron accumulation on the apical side of renal tubular cells in Heph/Cp KO mice...
December 19, 2016: Scientific Reports
https://read.qxmd.com/read/27893831/retinal-pre-conditioning-by-cd59a-knockout-protects-against-light-induced-photoreceptor-degeneration
#20
Delu Song, Brooks Wilson, Liangliang Zhao, Rupak Bhuyan, Mausumi Bandyopadhyay, Arkady Lyubarsky, Chen Yu, Yafeng Li, Levi Kanu, Takashi Miwa, Wen-Chao Song, Silvia C Finnemann, Bärbel Rohrer, Joshua L Dunaief
Complement dysregulation plays a key role in the pathogenesis of age-related macular degeneration (AMD), but the specific mechanisms are incompletely understood. Complement also potentiates retinal degeneration in the murine light damage model. To test the retinal function of CD59a, a complement inhibitor, CD59a knockout (KO) mice were used for light damage (LD) experiments. Retinal degeneration and function were compared in WT versus KO mice following light damage. Gene expression changes, endoplasmic reticulum (ER) stress, and glial cell activation were also compared...
2016: PloS One
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