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Seyhan Dikci, Emrah Öztürk, Penpe Gül Fırat, Turgut Yılmaz, Mehmet Çağatay Taşkapan, Saim Yoloğlu
OBJECTIVE: To investigate whether serum vitamin D levels have an effect on pseudoexfolation (PEX) glaucoma/syndrome development and on the control of glaucoma in these cases. METHOD: A total of 31 cases with PEX glaucoma, 34 cases with the PEX syndrome and 43 control subjects of similar age and sex were included in the study. Vitamin D levels were compared between the groups and also between the cases where glaucoma surgery was performed or not. RESULTS: PEX glaucoma group consisted of 17 males and 14 females, PEX syndrome group of 27 males and 7 females, and the control group of 27 males and 16 females...
November 27, 2018: Endocrine, Metabolic & Immune Disorders Drug Targets
Vishal C Kalel, Pascal Mäser, Michael Sattler, Ralf Erdmann, Grzegorz M Popowicz
Glycosomes evolved as specialized system for glycolysis in trypanosomatids. These organelle rely on protein import to maintain function. A machinery of peroxin (PEX) proteins is responsible for recognition and transport of glycosomal proteins to the organelle. Disruption of PEX-based import system was expected to be a strategy against trypanosomatids. Recently, a proof of this hypothesis has been presented. Here, we review current information about trypanosomatids' glycosomal transport components as targets for new trypanocidal therapies...
November 24, 2018: Current Opinion in Microbiology
Kak-Ming Ling, Luke W Garratt, Timo Lassmann, Stephen M Stick, Anthony Kicic
Chronic lung disease remains the primary cause of mortality in cystic fibrosis (CF). Growing evidence suggests respiratory viral infections are often more severe in CF compared to healthy peers and contributes to pulmonary exacerbations (PEx) and deterioration of lung function. Rhinovirus is the most prevalent respiratory virus detected, particularly during exacerbations in children with CF <5 years old. However, even though rhinoviral infections are likely to be one of the factors initiating the onset of CF lung disease, there is no effective targeted treatment...
2018: Frontiers in Pharmacology
Fritz H Hengerer, Gerd U Auffarth, Christoffer Riffel, Ina Conrad-Hengerer
INTRODUCTION: The aim of this study was to assess 36-month outcomes after cataract surgery and implantation of two second-generation trabecular micro-bypass stents (iStent inject, Glaukos Corporation, San Clemente, CA, USA) into eyes with predominantly primary open-angle glaucoma (POAG) or pseudoexfoliative glaucoma (PEX). METHODS: This prospective, non-randomized, consecutive cohort study included eyes with POAG (n = 60), PEX (n = 15), appositional narrow-angle (n = 4), pigmentary (n = 1), or neovascular (secondary) (n = 1) glaucoma and cataract requiring surgery...
December 2018: Ophthalmology and Therapy
Ling Qian, Shulin Yu, Zhen Chen, Zhiqiang Meng, Shenglin Huang, Peng Wang
Pancreatic cancer is one of the most aggressive human malignancies and is associated with a dismal prognosis, which can be contributed to its atypical symptoms, metastatic propensity, and significant chemoresistance. Emerging evidence shows that pancreatic cancer cell-derived exosomes (PEXs) play critical roles in tumorigenesis and tumor development, as they are involved in drug resistance, immune evasion and metabolic reprograming, and distant metastasis of pancreatic cancer. Their numerous differentially expressed and functional contents make PEXs promising screening tools and therapeutic targets, which require further exploration...
November 9, 2018: Biochimica et biophysica acta. Reviews on cancer
Patrick Zadravec, Barbara M Braunger, Benjamin Melzer, Markus Kroeber, Michael R Bösl, Herbert Jägle, Ursula Schlötzer-Schrehardt, Ernst R Tamm
Sequence variants in LOXL1 coding for the secreted enzyme lysyl oxidase homolog 1 (LOXL1) associate with pseudoexfoliation (PEX) syndrome, a condition that is characterized by the deposition of extracellular fibrillar PEX material in the anterior eye and other parts of the body. Since the specific role of LOXL1 in the pathogenesis of PEX is unclear, and an increase in its expression was reported for early stages of PEX syndrome, we generated and studied transgenic mice with ocular overexpression of its mouse ortholog Loxl1...
November 3, 2018: Experimental Eye Research
Francesca Di Cara, Richard A Rachubinski, Andrew J Simmonds
Peroxisomes are ubiquitous membrane-enclosed organelles involved in lipid processing and reactive oxygen detoxification. Mutations in human peroxisome biogenesis genes ( Peroxin , PEX or Pex ) cause developmental disabilities and often early death. Pex5 and Pex7 are receptors that recognize different peroxisomal targeting signals called PTS1 and PTS2, respectively, and traffic proteins to the peroxisomal matrix. We characterized mutants of Drosophila melanogaster Pex5 and Pex7 and found that adult animals are affected in lipid processing...
November 2, 2018: Genetics
Piotr Piejko, Agnieszka Ścisłowicz, Mateusz Nowak, Paulina Renke, Piotr Chaniecki
Pseudoexfoliation syndrome (PEX) is systematic microfibrylopathy associated with abnormalities of the accumulation of the extracellular matrix - buildup of protein clumps of abnormal basal membrane of trabecular epithelium, iris, ciliary body and lens capsule. The exfoliation material accumulates in anterior segment of a human eye (anterior lens capsule, ciliary zonule, ciliary body, iris, trabecular, anterior hyaloid membrane, conjunctiva) and other organs such as heart, connective tissue of lungs, vessels, skin, gallbladder, kidneys, liver, urinary bladder and meninges...
October 29, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
Gillian E MacLean, Catherine Argyriou, Erminia Di Pietro, Xuting Sun, Sara Birjandian, Panteha Saberian, Joseph G Hacia, Nancy E Braverman
Zellweger spectrum disorder (ZSD) results from biallelic mutations in PEX genes required for peroxisome biogenesis. PEX1-G843D is a common hypomorphic allele in the patient population that is associated with milder disease. In prior work using a PEX1-G843D/null patient fibroblast line expressing a green fluorescent protein (GFP) reporter with a peroxisome-targeting signal (GFP-PTS1), we demonstrated that treatments with the chemical chaperone betaine and flavonoid acacetin diacetate recovered peroxisome functions...
October 26, 2018: Journal of Cellular Biochemistry
Aravind Haripriya, Pradeep Y Ramulu, Shivkumar Chandrashekharan, Rengaraj Venkatesh, Kalpana Narendran, Madhu Shekhar, Rengappa Ramakrishnan, Ravilla D Ravindran, Alan L Robin
PURPOSE: To compare: (1) intraoperative complication rates, (2) one-year visual outcomes, and (3) post-operative complication rates over the first post-operative year in eyes with and without pseudoexfoliation (PEX) undergoing cataract surgery. DESIGN: Prospective comparative interventional study PARTICIPANTS: Nine-hundred thirty eyes with cataract and uncomplicated PEX (without phacodonesis, clinically shallow anterior chambers, or pupil size < 4mm ) and 476 controls with cataract but without PEX recruited from four centers of the Aravind Eye Care System in Southern India...
October 16, 2018: Ophthalmology
Ramazan Yağcı, Serdar Dervişoğulları, Emre Güler, Yüksel Totan, İbrahim Feyzi Hepşen
PURPOSE: The purpose of the present study was to evaluate the aqueous humor Endocan and Endostatin levels in patients with pseudoexfoliation syndrome (PEX) and to compare the results with healthy individuals. METHODS: 29 cataract patients with PEX syndrome (PEX group) and 32 cataract patients without PEX syndrome (control group) were enrolled in the study. Endocan and Endostatin were measured in the aqueous humor of the PEX and control groups by enzyme-linked immunosorbent assay...
October 18, 2018: Journal of Glaucoma
Stéphanie Romeo Villadóniga, Elena Rodríguez García, Olatz Sagastagoia Epelde, M Dolores Álvarez Díaz, Joan Carles Domingo Pedrol
Purpose: To assess the effects of antioxidant oral supplementation based on docosahexaenoic acid (DHA) in pseudoexfoliative (PEX) glaucoma. Patients and Methods: A prospective 6-month open-label randomized controlled trial was conducted in patients with PEX glaucoma and adequate intraocular pressure (IOP) control. Patients in the DHA group received a high-rich DHA (1 g) nutraceutical formulation. Ophthalmological examination, DHA erythrocyte membrane content (% total fatty acids), plasma total antioxidant capacity (TAC), plasma malondialdehyde (MDA), and plasma IL-6 levels were assessed...
2018: Journal of Ophthalmology
Yukichi Fujikawa, Marina Suekawa, Satoshi Endo, Youjirou Fukami, Shoji Mano, Mikio Nishimura, Muneharu Esaka
We evaluated the import of Arabidopsis catalase to peroxisomes under homogenous transient expression. The amino acids at -11 to -4 from the C-terminus are necessary for catalase import. The results are in agreement with the previous work under stable expression. We first demonstrate that heme-binding sites are important for peroxisomal import, suggesting the importance of catalase folding. ABBREVIATIONS: AtCat: Arabidopsis catalase; PTS: peroxisomal targeting signal; PEX: Peroxin.
October 8, 2018: Bioscience, Biotechnology, and Biochemistry
J E Cote-Orozco, O Y Echeverri-Pena, J M Guevara-Morales, E Espinosa
INTRODUCTION: Peroxisomal biogenesis disorders are due to mutations in the PEX genes, which code for peroxins that are required for peroxisomal biogenesis. Clinically, they are expressed as a Zellweger syndrome spectrum, and there is a wide phenotypic variety. They are diagnosed biochemically, and confirmation is molecular. The aim of this illustrative case is to highlight the importance of the clinical features and biochemical testing in the management of a peroxisomal disease. CASE REPORT: A 3-year-old boy with neonatal hypotonia, overall developmental delay and failure to thrive and a pattern of hypomyelinating leukodystrophy in brain resonance...
October 16, 2018: Revista de Neurologia
Curt Ekström, Sophia Wilger, Anders Wanhainen
PURPOSE: To study the relationship between pseudoexfoliation (PEX) and aortic aneurysm in a population with a high prevalence of PEX. METHODS: Survival analyses were performed in a cohort of 735 residents aged 65-74 years, examined in a population survey in the municipality of Tierp, Sweden, 1984-1986. To expand the sample size, 1040 people were recruited by means of glaucoma case records established at the Eye Department in Tierp in 1978-2007. In this way, the cohort comprised 1775 subjects, representing more than 25 400 person-years at risk...
September 20, 2018: Acta Ophthalmologica
Tanja Falter, Stephanie Herold, Veronika Weyer-Elberich, Carina Scheiner, Veronique Schmitt, Charis von Auer, Xavier Messmer, Philipp Wild, Karl J Lackner, Bernhard Lämmle, Inge Scharrer
BACKGROUND:  Autoimmune thrombotic thrombocytopenic purpura (iTTP) is caused by autoantibody-mediated severe a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13) deficiency leading to micro-angiopathic haemolytic anaemia (MAHA) and thrombocytopenia with organ damage. Patients survive with plasma exchange (PEX), fresh frozen plasma replacement and corticosteroid treatment. Anti-CD20 monoclonal antibody rituximab is increasingly used in patients resistant to conventional PEX or relapsing after an acute bout...
October 2018: Thrombosis and Haemostasis
Alberto Galvez-Ruiz, Alicia Galindo-Ferreiro, Hind Alkatan
Peroxisomal biogenesis disorders (PBDs) are autosomal recessive diseases caused by mutations in one of the 14 PEX genes described in the scientific literature. All of these syndromes may be associated with different mutations in the PEX genes, the most frequent being PEX1 for patients with Zellweger syndrome (ZS). In this paper, we present the case of a patient with a peculiar clinical history: evisceration of the left eye (LE) at 4 years of age because of a benign ocular teratoid medulloepithelioma and a progressive loss of visual acuity (VA) in the right eye (RE) beginning at 9 years of age, leading to the diagnosis of ZS...
July 2018: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
C Lehoux Dubois, E Labrèche, V Boudreau, J Colomba, M Mailhot, A Lavoie, R Rabasa-Lhoret, A Coriati
BACKGROUND: Due to lack of vitamin D absorption in patients with cystic fibrosis (CF), vitamin D supplementation becomes necessary. Our aim was to study the association between serum vitamin D levels and key clinical factors, such as nutritional status, pulmonary function and pulmonary exacerbations (PEx) frequency, in an adult CF population. METHODS: Prospective analysis of a published vitamin D (VitD3 ) supplementation protocol (N = 200 adult patients) over a follow-up period of 5 years...
August 25, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
Steven P Campbell, Erin R Zylstra, Catherine R Darst, Roy C Averill-Murray, Robert J Steidl
Many of the processes that govern the viability of animal populations vary spatially, yet population viability analyses (PVAs) that account explicitly for spatial variation are rare. We develop a PVA model that incorporates autocorrelation into the analysis of local demographic information to produce spatially explicit estimates of demography and viability at relatively fine spatial scales across a large spatial extent. We use a hierarchical, spatial autoregressive model for capture-recapture data from multiple locations to obtain spatially explicit estimates of adult survival (ϕad ), juvenile survival (ϕjuv ), and juvenile-to-adult transition rates (ψ), and a spatial autoregressive model for recruitment data from multiple locations to obtain spatially explicit estimates of recruitment (R)...
September 5, 2018: Ecological Applications: a Publication of the Ecological Society of America
Laura S Gold, Donald L Patrick, Ryan N Hansen, Valeria Beckett, Christopher H Goss, Larry Kessler
BACKGROUND: Pulmonary exacerbations (PEx) in cystic fibrosis (CF) patients decrease lung function, increase symptoms and reduce health-related quality of life (HRQoL). We evaluated associations between 8 symptom-based questions from the Cystic Fibrosis Respiratory Symptom Diary - Chronic Respiratory Infection Symptom Score (CFRSD-CRISS) and the 5-level EuroQOL-5 Dimensions (EQ-5D-5 L) summary score and hypothesized the CFRSD-CRISS would be well-correlated with quality-of-life measures among CF patients with PEx...
August 28, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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