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https://www.readbyqxmd.com/read/29044065/the-adverse-effects-of-valproic-acid-on-visual-functions-in-the-treatment-of-retinitis-pigmentosa
#1
Yüksel Totan, Emre Güler, Aslıhan Yüce, Mehmet Serdar Dervişogulları
PURPOSE: To evaluate the efficacy and safety of valproic acid (VPA) treatment in patients with retinitis pigmentosa (RP). METHODS: A total of 48 eyes of 24 patients (13 males, 11 females) with RP prescribed VPA were included. The length of VPA treatment was 6-12 months (mean 9.4 months). Parameters evaluated were best-corrected visual acuity (BCVA) (logarithm of the minimum angle of resolution [logMAR]), visual field analyses (VFAs) with Humprey automated perimetry, multifocal electroretinography (ERG) with Roland-RETI scan, and VPA side effects...
October 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/29038003/hemin-supports-the-survival-of-photoreceptors-injured-by-n-methyl-n-nitrosourea-the-contributory-role-of-neuroglobin-in-photoreceptor-degeneration
#2
Ye Tao, Zhao Ma, Bei Liu, Wei Fang, Limin Qin, Yi Fei Huang, Lu Wang, Yanling Gao
Retina is a critical component of the central nerve system that is responsible for the conversion of light stimulus into electrical spikes. Retinitis pigmentosa (RP) comprises a heterogeneous group of inherited retinal dystrophies leading to blindness. We examined retinal neuroglobin (Ngb) expression in a pharmacologically induced RP animal model, the N-Methyl-N-nitrosourea (MNU) administered mice. The retinal Ngb expression in MNU administered mice attenuated following a time dependent manner, suggesting Ngb was involved in the photoreceptor degeneration...
October 13, 2017: Brain Research
https://www.readbyqxmd.com/read/29037514/re-association-between-combined-tmprss2-erg-and-pca3-rna-urinary-testing-and-detection-of-aggressive-prostate-cancer
#3
David G McLeod, Gyorgy Petrovics
No abstract text is available yet for this article.
October 13, 2017: European Urology
https://www.readbyqxmd.com/read/29036441/the-role-of-the-er-stress-response-protein-perk-in-rhodopsin-retinitis-pigmentosa
#4
Dimitra Athanasiou, Monica Aguila, James Bellingham, Naheed Kanuga, Peter Adamson, Michael E Cheetham
Mutations in rhodopsin, the light sensitive protein of rod cells, are the most common cause of dominant retinitis pigmentosa (RP), a type of inherited blindness caused by the dysfunction and death of photoreceptor cells. The P23H mutation, the most frequent single cause of RP in the USA, causes rhodopsin misfolding and induction of the unfolded protein response (UPR), an adaptive ER stress response and signalling network that aims to enhance the folding and degradation of misfolded proteins to restore proteostasis...
September 27, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/29029550/b-lymphoblastic-leukemia-lymphoma-new-insights-into-genetics-molecular-aberrations-subclassification-and-targeted-therapy
#5
REVIEW
Xiaohui Zhang, Prerna Rastogi, Bijal Shah, Ling Zhang
B lymphoblastic leukemia/lymphoma (B-ALL) is a clonal hematopoietic stem cell neoplasm derived from B-cell progenitors, which mostly occurs in children and adolescents and is regarded as one of top leading causes of death related to malignancies in this population. Despite the majority of patients with B-ALL have fairly good response to conventional chemotherapeutic interventions followed by hematopoietic stem cell transplant for the last decades, a subpopulation of patients show chemo-resistance and a high relapse rate...
September 12, 2017: Oncotarget
https://www.readbyqxmd.com/read/29029471/protective-effect-of-resveratrol-against-light-induced-retinal-degeneration-in-aged-samp8-mice
#6
Zhirong Liu, Zhengzheng Wu, Jie Li, Anna Marmalidou, Ruifan Zhang, Man Yu
PURPOSE: The purpose of this study was to determine the protective effects of Resveratrol (RESV) on acute bright light-induced retinal degeneration in aged senescence accelerated mouse strain. METHODS: Ten three-month-old male SAMP8 mice (prone to aging) were randomly assigned to two experimental dietary groups: one untreated group and one RESV treatment group (n=20 eyes for each group). After 30 days of treatment, mice were exposed to intense bright light. Ten male SAMR1 mice (resistant to aging) served as control (n=20 eyes)...
September 12, 2017: Oncotarget
https://www.readbyqxmd.com/read/29029032/foxa1-expression-is-a-strong-independent-predictor-of-early-psa-recurrence-in-erg-negative-prostate-cancers-treated-by-radical-prostatectomy
#7
Maria Christina Tsourlakis, Agapi Eleftheriadou, Annegret Stender, Philipp Weigand, Katharina Grupp, Claudia Hube-Magg, Martina Kluth, Cornelia Schroeder, Stefan Steurer, Andrea Hinsch, Andreas Luebke, Alexander Angerer, Corinna Wittmer, Emily Friedrich, Cosima Göbel, Franziska Büscheck, Hans Heinzer, Markus Graefen, Ronald Simon, Guido Sauter, Waldemar Wilczak, Sarah Minner, Thorsten Schlomm, Frank Jacobsen
FOXA1 is a transcription factor involved in androgen signaling with relevance for lineage specific gene expression of the prostate. The expression was analyzed by immunohistochemistry on a tissue microarray containing 11,152 prostate cancer specimens. Results were compared to tumor phenotype, biochemical recurrence, androgen receptor expression, ERG status and other recurrent genomic alterations. FOXA1 expression was detectable in 97.6% of 8,227 interpretable cancers and considered strong in 28.5%, moderate in 46...
September 26, 2017: Carcinogenesis
https://www.readbyqxmd.com/read/29026072/dynamic-regulation-of-canonical-tgf%C3%AE-signalling-by-endothelial-transcription-factor-erg-protects-from-liver-fibrogenesis
#8
Neil P Dufton, Claire R Peghaire, Lourdes Osuna-Almagro, Claudio Raimondi, Viktoria Kalna, Abhishek Chuahan, Gwilym Webb, Youwen Yang, Graeme M Birdsey, Patricia Lalor, Justin C Mason, David H Adams, Anna M Randi
The role of the endothelium in protecting from chronic liver disease and TGFβ-mediated fibrosis remains unclear. Here we describe how the endothelial transcription factor ETS-related gene (ERG) promotes liver homoeostasis by controlling canonical TGFβ-SMAD signalling, driving the SMAD1 pathway while repressing SMAD3 activity. Molecular analysis shows that ERG binds to SMAD3, restricting its access to DNA. Ablation of ERG expression results in endothelial-to-mesenchymal transition (EndMT) and spontaneous liver fibrogenesis in EC-specific constitutive hemi-deficient (Erg (cEC-Het) ) and inducible homozygous deficient mice (Erg (iEC-KO) ), in a SMAD3-dependent manner...
October 12, 2017: Nature Communications
https://www.readbyqxmd.com/read/29025171/-the-impact-of-macular-development-on-full-field-and-multifocal-erg-in-extremely-preterm-born-children-with-and-without-acute-retinopathy-of-prematurity
#9
Wadim Bowl, Silke Schweinfurth, Kerstin Holve, Knut Stieger, Birgit Lorenz, Monika Andrassi-Darida
Introduction Retinal development is a complex process that can continue into early childhood and beyond. Prematurity can affect the maturation of the central retina, characterised by a flatter foveal pit and overlying inner retinal layers (IRL), leading to a disturbed ratio of outer retinal layers to IRL ("macular developmental arrest": MDA) and functional impairment (Bowl et al. 2016 18). The purpose of this study was to correlate functional results by electrophysiology with the morphological appearance of the fovea in children with spontaneously regressed and without ROP and term-born age-matched controls...
October 2017: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/28993665/variabilities-in-retinal-function-and-structure-in-a-canine-model-of-cone-rod-dystrophy-associated-with-rpgrip1-support-multigenic-etiology
#10
Rueben G Das, Felipe Pompeo Marinho, Simone Iwabe, Evelyn Santana, Kendra Sierra McDaid, Gustavo D Aguirre, Keiko Miyadera
Defects in the cilia gene RPGRIP1 cause Leber congenital amaurosis and cone-rod dystrophy in humans. A form of canine cone-rod dystrophy (cord1) was originally associated with a homozygous insertion in RPGRIP1 (RPGRIP1 (ins/ins)) as the primary disease locus while a homozygous deletion in MAP9 (MAP9 (del/del)) was later identified as a modifier associated with the early onset form. However, we find further variability in cone electroretinograms (ERGs) ranging from normal to absent in an extended RPGRIP1 (ins/ins) canine colony, irrespective of the MAP9 genotype...
October 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28986597/associations-between-outer-retinal-structures-and-focal-macular-electroretinograms-in-patients-with-retinitis-pigmentosa
#11
Taro Kominami, Shinji Ueno, Azusa Kominami, Ayami Nakanishi, Shunsuke Yasuda, Chang-Hua Piao, Satoshi Okado, Hiroko Terasaki
Purpose: Our earlier study showed that the width of the intact ellipsoid zone (EZ) of the photoreceptors was significantly but weakly correlated with the amplitudes of the focal macular ERGs (FMERGs). The aim of this study was to determine a microstructure of the photoreceptors in the spectral-domain optical coherence tomographic (SD-OCT) images that was more strongly correlated with the FMERG parameters in eyes with retinitis pigmentosa (RP). Methods: This was a retrospective, observational study...
October 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28986382/loss-of-foxo1-cooperates-with-tmprss2-erg-overexpression-to-promote-prostate-tumorigenesis-and-cell-invasion
#12
Yinhui Yang, Alexandra M Blee, Dejie Wang, Jian An, Yunqian Pan, Yuqian Yan, Tao Ma, Yundong He, Joseph Dugdale, Xiaonan Hou, Jun Zhang, S John Weroha, Wei-Guo Zhu, Y Alan Wang, Ronald A DePinho, Wanhai Xu, Haojie Huang
E26 transformation-specific (ETS) transcription factor ERG is aberrantly overexpressed in approximately 50% of all human PCa due to TMPRSS2-ERG gene rearrangements. However, mice with prostate-specific transgenic expression of PCa-associated ERG alone fail to develop PCa, highlighting that ERG requires other lesions to drive prostate tumorigenesis. Forkhead box (FOXO) transcription factor FOXO1 is a tumor suppressor that is frequently inactivated in human PCa. Here we demonstrate that FOXO1, but not other FOXO proteins (FOXO3 and FOXO4), binds and inhibits the transcriptional activity of PCa-associated ERG independently of FOXO1 transcriptional activity...
October 6, 2017: Cancer Research
https://www.readbyqxmd.com/read/28986343/treatment-of-experimental-autoimmune-uveoretinitis-with-intravitreal-injection-of-infliximab-encapsulated-in-liposomes
#13
Rui Zhang, Jiang Qian, Xiaofeng Li, Yifei Yuan
AIMS: To evaluate the safety and efficacy of intravitreal injection of liposomes encapsulating infliximab in experimental autoimmune uveoretinitis (EAU) rats. METHODS: Liposomes containing infliximab were prepared and characterised for mean particle size, entrapment efficiency, polydispersity index (PDI) and zeta potential. In vitro release profile and the stability of infliximab-lip were evaluated. EAU rats were intravitreally injected with saline, infliximab, infliximab-lip or unloaded liposomes...
October 6, 2017: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/28979999/therapeutic-effects-of-a-novel-agonist-of-peroxisome-proliferator-activated-receptor-alpha-for-the-treatment-of-diabetic-retinopathy
#14
Guotao Deng, Elizabeth P Moran, Rui Cheng, Greg Matlock, Kelu Zhou, David Moran, Danyang Chen, Qiang Yu, Jian-Xing Ma
Purpose: Clinical studies have shown that peroxisome proliferator-activated receptor alpha (PPARα) agonist fenofibrate has therapeutic effects on diabetic retinopathy (DR). The purpose of this study was to identify a novel PPARα agonist and to evaluate its beneficial effects on DR. Methods: The transcriptional activity of PPARα was measured by a luciferase-based promoter assay. TUNEL was used to evaluate apoptosis in retinal precursor cells (R28). Diabetes was induced in rats by injection of streptozotocin...
October 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28978861/acute-leukemia-in-adolescents-and-young-adults
#15
Daisuke Tomizawa
Both acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) are common malignant diseases in adolescents and young adults (AYAs). Recent advances in genomic studies have helped us understand the biological nature of acute leukemia in AYAs; higher frequency of Ph-like ALL and rearrangements in DUX4, ERG, MEF2D, and ZNF384 genes in AYAs with ALL and higher frequency of FLT3-ITD, NPM1, IDH1/2, DNMT3A, ASXL1, TET2, and CEBPA mutations in AYAs with AML than that in children. The pediatric-inspired regimen has become a standard treatment approach for AYAs with ALL, but optimal treatment strategy for AYAs with AML is not yet established to date...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28977868/thioredoxin-plays-a-key-role-in-retinal-neuropathy-prior-to-endothelial-damage-in-diabetic-mice
#16
Xiang Ren, Chen Li, Junli Liu, Chenghong Zhang, Yuzhen Fu, Nina Wang, Haiying Ma, Heyuan Lu, Hui Kong, Li Kong
Diabetes is a chronic metabolic syndrome that results in changes in carbohydrate, lipid and protein metabolism. With diabetes for a long time, it increases the risk of diabetic retinopathy (DR) and long-term morbidity and mortality. Moreover, emerging evidence suggests that neuron damage occurs earlier than microvascular complications in DR patients, but the underlying mechanism is unclear. We investigated diabetes-induced retinal neuropathy and elucidated key molecular events to identify new therapeutic targets for the clinical treatment and prevention of DR...
September 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/28975461/measurement-of-dark-adaptometry-during-iscev-standard-flash-electroretinography
#17
Jeff Rabin, Brooke Houser, Carolyn Talbert, Rue Patel
PURPOSE: Dark adaptometry (DA) is a sensitive test for diagnosis of retinal disease. Patients requiring flash electroretinograms (ERGs) benefit from DA for proper diagnosis. Our purpose was to develop a DA test to be administered during the 20-min dark adaptation period of the flash ERG ( www.iscev.org ) using alternating red and blue stimuli to bias responses in favor of cones (red) and rods (blue). METHODS: An ERG Ganzfeld was used to measure DA in 21 normal subjects and 21 patients with retinal disease...
October 3, 2017: Documenta Ophthalmologica. Advances in Ophthalmology
https://www.readbyqxmd.com/read/28974978/can-allopurinol-improve-retinopathy-in-diabetic-rats-oxidative-stress-or-uric-acid-which-one-is-the-culprit
#18
Mohsen Goharinia, Athar Zareei, Mansour Rahimi, Hossein Mirkhani
Allopurinol, an inhibitor of xanthine oxidase, reduces both plasma uric acid and oxidative stress and shows useful effects on some complications of diabetes. However, it is not defined which of the above mentioned properties are involved. Moreover, to the best of our knowledge no study has been done on the effects of allopurinol on diabetic retinopathy. In the present study, the effect of allopurinol on experimental diabetic retinopathy and its possible mechanism has been investigated. Thirty two rats were divided into four groups of eight rats each; (1) normal, (2) diabetic control, (3) diabetic + allopurinol (50 mg/kg...
October 2017: Research in Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28974356/long-term-photoreceptor-rescue-in-two-rodent-models-of-retinitis-pigmentosa-by-adeno-associated-virus-delivery-of-stanniocalcin-1
#19
Gavin W Roddy, Douglas Yasumura, Michael T Matthes, Marcel V Alavi, Sanford L Boye, Robert H Rosa, Michael P Fautsch, William W Hauswirth, Matthew M LaVail
Retinal degenerations, including age-related macular degeneration and the retinitis pigmentosa family of diseases, are among the leading causes of legal blindness in the United States. We previously found that Stanniocalcin-1 (STC-1) reduced photoreceptor loss in the S334ter-3 and Royal College of Surgeons rat models of retinal degeneration. The results were attributed in part to a reduction in oxidative stress. Herein, we tested the hypothesis that long-term delivery of STC-1 would provide therapeutic rescue in more chronic models of retinal degeneration...
September 30, 2017: Experimental Eye Research
https://www.readbyqxmd.com/read/28968266/utility-of-erg-immunohistochemistry-for-evaluation-of-lymphovascular-invasion-in-testicular-germ-cell-tumors-a-retrospective-pilot-study
#20
Aaron M Udager, Jonathan B McHugh, Todd M Morgan, Daniel E Spratt, Arul M Chinnaiyan, Rohit Mehra
Lymphovascular invasion (LVI) of testicular germ cell tumors (GCT) is an important stage-determining variable in the evaluation of radical orchiectomy specimens. ERG endothelial cell expression, as detected by immunohistochemistry (IHC), robustly highlights lymphovascular spaces, and thus, we sought to assess the utility of ERG IHC for evaluation of GCT LVI. Hematoxylin and eosin (H&E) slides from a retrospective cohort of 25 GCT radical orchiectomy specimens (emanating from a parent cohort of 159 radical orchiectomy GCT cases identified between 2003 and 2013) were reviewed, and sections with foci of positive or equivocal LVI were identified...
September 29, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
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