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https://www.readbyqxmd.com/read/29116306/short-mood-and-feelings-questionnaire-for-screening-children-and-adolescents-for-plastic-surgery-cross-cultural-validation-study
#1
Eduardo Sucupira, Miguel Sabino, Edson Luiz de Lima, Gal Moreira Dini, Maria José Azevedo de Brito, Lydia Masako Ferreira
CONTEXT AND OBJECTIVE: Patient-reported outcome measurements assessing the emotional state of children and adolescents who seek plastic surgery are important for determining whether the intervention is indicated or not. The aim of this study was to cross-culturally adapt and validate the Short Mood and Feelings Questionnaire (child/adolescent and parent versions) for Brazilian Portuguese, test its psychometric properties and assess the emotional state of children and adolescents who seek plastic surgery...
November 6, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/29099167/separation-and-analysis-of-lactosylceramide-galabiosylceramide-and-globotriaosylceramide-by-lc-ms-ms-in-urine-of-fabry-disease-patients
#2
Michel Boutin, Iskren Menkovic, Tristan Martineau, Vanessa Vaillancourt-Lavigueur, Amanda Toupin, Christiane Auray-Blais
Fabry disease is an X-linked lysosomal storage disorder caused by alpha-galactosidase A (α-GAL A) deficiency. This enzyme contributes to the cellular recycling of glycosphingolipids such as galabiosylceramide (Ga2), globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3) by hydrolysing the terminal alpha-galactosyl moiety. Urine and plasma α-GAL A substrates are currently analyzed as biomarkers for the detection, monitoring and follow-up of Fabry disease patients. The sensitivity of the analysis of Ga2 is decreased by the co-analysis of its structural isomer, lactosylceramide (LacCer), which is not an α-GAL A substrate...
November 3, 2017: Analytical Chemistry
https://www.readbyqxmd.com/read/29074067/a-tick-acquired-red-meat-allergy-a-case-series
#3
Joe Kevin Khoury, Neil Christian Khoury, Deborah Schaefer, Anup Chitnis, Getaw Worku Hassen
Allergic reaction is a common clinical picture in the Emergency Department (ED). Most allergic reactions are from food or drugs. A detailed history is an integral aspect of determining the causative agent of an allergy. Galactose-alpha-1,3-galactose (alpha-gal) allergy is a tick-acquired red meat allergy that causes delayed-onset allergic reaction or anaphylaxis due to molecular mimicry. Alpha-gal allergy may not be widely known as a cause of allergic reactions. Lack of universal awareness of this phenomenon in the ED and Urgent Care setting could lead to misdiagnosis, or delayed diagnosis...
October 16, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29057501/different-approaches-to-heart-valve-decellularization-a-comprehensive-overview-of-the-past-30%C3%A2-years
#4
REVIEW
Filippo Naso, Alessandro Gandaglia
Xenogeneic decellularized heart valve scaffolds have the potential to overcome the limitations of existing bioprosthetic heart valves that have limited duration due to calcification and tissue degeneration phenomena. This article presents a review of 30 years of decellularization approaches adopted in cardiovascular tissue engineering, with a focus on the use, either individually or in combination, of different detergents. The safety and efficacy of cell-removal procedures are specifically reported and discussed, as well as the structure and biomechanics of the treated extracellular matrix (ECM)...
October 22, 2017: Xenotransplantation
https://www.readbyqxmd.com/read/29053434/alpha-gal-inactivated-heart-valve-bioprostheses-exhibit-an-anti-calcification-propensity-similar-to-knockout-tissues
#5
Filippo Naso, Ugo Stefanelli, Edward Buratto, Giovanna Lazzari, Andrea Perota, Cesare Galli, Alessandro Gandaglia
BACKGROUND: Glutaraldehyde (GLA) has been used to crosslink bioprosthetic heart valve (BHVs) tissues to enhance their stability, besides ensuring a satisfactory degree of immunological tolerance. Unfortunately, GLA fixation does not guarantee a complete tissue biocompatibility of BHVs in currently used devices. The interaction between preformed human anti-alpha-Gal antibody and alpha-Gal antigens promotes the calcification of GLA-treated alpha-Gal-positive tissue. Recently, an alarming correlation between the presence of the alpha-Gal epitope and a premature BHVs degeneracy was reported...
October 2017: Tissue Engineering. Part A
https://www.readbyqxmd.com/read/29044343/case-report-is-low-%C3%AE-gal-enzyme-activity-sufficient-to-establish-the-diagnosis-of-fabry-disease
#6
Gilson Biagini, Ana Clara Simões Flórido Almeida, Tammy Vernalha Rocha Almeida, Cassiano Augusto Braga Silva, Bruna Fernanda de Castro, Tais Cristina Reche, Ana Cláudia Dabinski, Fellype Carvalho Barreto
Fabry disease is an X-linked lysosomal storage disease due to alpha-galactosidase A (α-Gal A) deficient activity which leads to the accumulation of glucoesphingolipids, such as globotriaosilceramide. There are over 700 known mutations of the enzyme gene, and most of them cause Fabry Disease. This case report describes a hemodialysis patient with a rare and controversial GLA gene mutation, the D313Y. The medecial investigation confirmed that D313Y is an alpha-galactosidase A sequence variant that causes pseudo deficient enzyme activity in plasma but not Fabry disease...
July 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29037082/the-d313y-variant-in-the-gla-gene-no-evidence-of-a-pathogenic-role-in-fabry-disease
#7
Lis Hasholt, Martin Ballegaard, Henning Bundgaard, Michael Christiansen, Ian Law, Allan M Lund, Anne Norremolle, Ase Krogh Rasmussen, Kirstine Ravn, Zeynep Tumer, Flemming Wibrand, Ulla Feldt-Rasmussen
Fabry disease is an X- linked inherited lysosomal storage disease caused by mutations in the GLA gene encoding the lysosomal enzyme alpha-galactosidase A (α-Gal A). The possible pathological significance of the D313Y variant in the GLA gene has not been verified and it may be a Fabry variant. Our aim was to elucidate whether the presence of the D313Y variant influenced the α-Gal A activity or resulted in Fabry symptoms or Fabry organ involvement. In two Danish families the presence of the D313Y variant did not result in reduced α-Gal A activity or clinical Fabry manifestations in males, and the presence in Fabry females did not significantly enhance the phenotype of a known causative mutation in the GLA gene (G271S)...
October 16, 2017: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/29019163/enzyme-replacement-therapy-in-a-patient-of-heterozygous-fabry-disease-clinical-and-pathological-evaluations-by-repeat-kidney-biopsy-and-a-successful-pregnancy
#8
Yoichi Iwafuchi, Hiroki Maruyama, Tetsuo Morioka, Seiko Noda, Hiroshi Nagata, Yuko Oyama, Ichiei Narita
Fabry disease is a rare X-linked lysosomal storage disorder of glycosphingolipid catabolism caused by deficient activity of the lysosomal hydrolase alpha-galactosidase A (ɑ-Gal A). A 20-year-old woman was referred to our hospital because of proteinuria and persistent macroscopic hematuria. Based on the typical renal pathological findings, deficient activity of the ɑ-Gal A, and heterozygous mutation in the ɑ-Gal A gene, she was diagnosed with Fabry disease. After 1 year of enzyme replacement therapy with agalsidase alfa at 0...
October 10, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28972138/photorhabdus-luminescens-lectin-a-plla-a-new-probe-for-detecting-%C3%AE-galactoside-terminating-glycoconjugates
#9
Ghamdan Beshr, Asfandyar Sikandar, Eva-Maria Jemiller, Nikolai Klymiuk, Dirk Hauck, Stefanie Wagner, Eckhard Wolf, Jesko Koehnke, Alexander Titz
Lectins play important roles in infections by pathogenic bacteria, for example, in host colonization, persistence and biofilm formation. The Gram-negative entomopathogenic bacterium Photorhabdus luminescens symbiotically lives in insect-infecting Heterorhabditis nematodes and kills the insect host upon invasion by the nematode. The P. luminescens genome harbors the gene plu2096 coding for a novel lectin that we named PllA. We analyzed the binding properties of purified PllA with a glycan array and a binding assay in solution...
September 28, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28932561/the-predictive-value-of-plasma-cytokines-on-gastroesophageal-anastomotic-leakage-at-an-early-stage-in-patients-undergoing-esophagectomy
#10
Jie-Qiong Song, Yi-Zhou He, Yuan Fang, Wei Wu, Ming Zhong
BACKGROUND: It's difficult to diagnose gastroesophageal anastomotic leakage (GAL) at early postoperative stage. This study was conducted to evaluate the early predictive value of plasma cytokines levels on GAL in patients undergoing esophagectomy. METHODS: Consecutive esophageal cancer patients who underwent esophagectomy and admitted to Surgical Intensive Care Unit (SICU) just after surgery were retrospectively analyzed. The baseline and postoperative 1 day plasma cytokine levels were collected and analyzed to evaluate the predictive value for clinically important anastomotic leakage...
August 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28921428/production-of-recombinant-factor-viii-in-human-cell-lines
#11
Daianne Maciely Carvalho Fantacini, Virgínia Picanço-Castro
Human cell lines can produce recombinant proteins much more similar to their natural counterpart, compared to other mammalian cell lines, reducing potential immunogenic reactions. Recombinant proteins produced in nonhuman cells can have in its structure glycan epitopes, such as Galα1,3-Gal (alpha-Gal) and N-glycolylneuraminic acid (Neu5Gc) residues, that are antigenic to humans and can potentially affect the efficacy of the recombinant product. Therefore, the production of recombinant factor VIII (rFVIII) in human cell lines is a new approach to avoid nonhuman glycosylation...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28890075/down-regulation-of-dorsal-striatal-%C3%AE-camkii-causes-striatum-related-cognitive-and-synaptic-disorders
#12
Qi Wang, Pengcheng Yin, Bin Yu, Zheng Zhao, Gal Richter-Levin, Lu Yu, Xiaohua Cao
Alpha calcium/calmodulin dependent protein kinase II (αCaMKII) is a serine/threonine protein kinase which is expressed abundantly in dorsal striatum and is highly involved in the corticostriatal synaptic plasticity. Nevertheless, it currently remains unclear whether and how αCaMKII plays a in the striatum-related neural disorders. To address the above issue, lentivirus-mediated short hairpin RNA (shRNA) was used to silence the expression of αCaMKII gene in the dorsal striatum of mice. As a consequence of down-regulation of dorsal striatal αCaMKII expression, we observed defective motor skill learning in accelerating rotarod and response learning in water cross maze...
December 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28842010/a-novel-high-throughput-screening-assay-for-binding-affinities-of-perfluoroalkyl-iodide-for-estrogen-receptor-alpha-and-beta-isoforms
#13
Wenting Song, Lixia Zhao, Zhendong Sun, Xiaoxi Yang, Qunfang Zhou, Guibin Jiang
Contaminants of emerging concern are continuously increasing, which makes it important to develop high throughput screening techniques for the evaluation of their potential biological effects, especially endocrine disrupting effects, which would directly influence the population dynamics in environment. A novel competitive binding assay based on enzyme fragmentation complementation technology was established to screen the binding affinities of emerging chemicals for estrogen receptor (ER) α or β isoforms...
December 1, 2017: Talanta
https://www.readbyqxmd.com/read/28815010/development-and-characterisation-of-a-low-concentration-sodium-dodecyl-sulphate-decellularised-porcine-dermis
#14
Jack A Helliwell, Daniel S Thomas, Vaia Papathanasiou, Shervanthi Homer-Vanniasinkam, Amisha Desai, Louise M Jennings, Paul Rooney, John N Kearney, Eileen Ingham
The aim of this study was to adapt a proprietary decellularisation process for human dermis for use with porcine skin. Porcine skin was subject to: sodium chloride (1 M) to detach the epidermis, trypsin paste to remove hair follicles, peracetic acid (0.1% v/v) disinfection, washed in hypotonic buffer and 0.1% (w/v) sodium dodecyl sulphate in the presence of proteinase inhibitors followed by nuclease treatment. Cellular porcine skin, decellularised porcine and human dermis were compared using histology, immunohistochemistry, GSL-1 lectin (alpha-gal epitope) staining, biochemical assays, uniaxial tensile and in vitro cytotoxicity tests...
January 2017: Journal of Tissue Engineering
https://www.readbyqxmd.com/read/28808181/screening-of-fabry-disease-in-patients-with-end-stage-renal-disease-of-unknown-etiology-the-first-thailand-study
#15
Objoon Trachoo, Paisan Jittorntam, Sarunpong Pibalyart, Saowanee Kajanachumphol, Norasak Suvachittanont, Suthep Patputthipong, Piyatida Chuengsaman, Arkom Nongnuch
We aimed to explore the prevalence of Fabry disease in Thai patients who were diagnosed with end-stage renal disease (ESRD) of an unknown origin. Venous blood samples were collected from ESRD patients for biochemical and molecular studies. Alpha-galactosidase A (α-GAL A) screening was performed from dried-blood spots using fluorometry. Molecular confirmation was performed using DNA sequencing of the GLA gene. A total of 142 male and female patients were included in this study. Ten patients (7.04%) exhibited a significant decrease in α-GAL A activity...
October 17, 2016: Journal of Biomedical Research
https://www.readbyqxmd.com/read/28752723/red-meat-allergy-induced-by-tick-bites-a-norwegian-case-report
#16
G Arslan Lied
Food allergies, especially delayed hypersensitivity reactions, are often challenging for both patients and clinicians. Here, we report the case of a 64-year-old man who had allergic reactions six hours after eating a meal containing red meat. He reported that he had several tick bites in months before the reaction. High serum specific IgE levels of alpha-gal confirmed the diagnosis of alpha-gal allergic reaction with delayed onset after red meat ingestion caused by tick bite.
July 2017: European Annals of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28725181/impact-of-proestrus-on-gene-expression-in-the-medial-preoptic-area-of-mice
#17
Csaba Vastagh, Zsolt Liposits
The antero-ventral periventricular zone (AVPV) and medial preoptic area (MPOA) have been recognized as gonadal hormone receptive regions of the rodent brain that-via wiring to gonadotropin-releasing hormone (GnRH) neurons-contribute to orchestration of the preovulatory GnRH surge. We hypothesized that neural genes regulating the induction of GnRH surge show altered expression in proestrus. Therefore, we compared the expression of 48 genes obtained from intact proestrous and metestrous mice, respectively, by quantitative real-time PCR (qPCR) method...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28680430/human-alpha-galactosidases-transiently-produced-in-nicotiana-benthamiana-leaves-new-insights-in-substrate-specificities-with-relevance-for-fabry-disease
#18
Kassiani Kytidou, Thomas J M Beenakker, Lotte B Westerhof, Cornelis H Hokke, Geri F Moolenaar, Nora Goosen, Mina Mirzaian, Maria J Ferraz, Mark de Geus, Wouter W Kallemeijn, Herman S Overkleeft, Rolf G Boot, Arjen Schots, Dirk Bosch, Johannes M F G Aerts
Deficiency of α-galactosidase A (α-GAL) causes Fabry disease (FD), an X-linked storage disease of the glycosphingolipid globtriaosylcerammide (Gb3) in lysosomes of various cells and elevated plasma globotriaosylsphingosine (Lyso-Gb3) toxic for podocytes and nociceptive neurons. Enzyme replacement therapy is used to treat the disease, but clinical efficacy is limited in many male FD patients due to development of neutralizing antibodies (Ab). Therapeutic use of modified lysosomal α-N-acetyl-galactosaminidase (α-NAGAL) with increased α-galactosidase activity (α-NAGAL(EL)) has therefore been suggested...
2017: Frontiers in Plant Science
https://www.readbyqxmd.com/read/28670695/meat-allergy-associated-with-%C3%AE-gal-closing-diagnostic-gaps-by-anti-%C3%AE-gal-ige-immune-profiling
#19
U Jappe, S Minge, B Kreft, A Ludwig, B Przybilla, A Walker, R Varga, P Seidel, T Biedermann, W Anemüller, A Kromminga, F Ruëff, H Merk, N Wagner, R Treudler, M Worm, I Waldmann, J Saloga, W M Becker, T Goldmann, T A Platts-Mills, A Homann
BACKGROUND: Glycoproteins and glycolipids of some mammalian species contain the disaccharide galactosyl-α-(1,3)-galactose (α-Gal). It is known that α-Gal is immunogenic in humans and causes glycan-specific IgG and also IgE responses with clinical relevance. α-Gal is part of the IgE-reactive monoclonal therapeutic antibody cetuximab and is associated with delayed anaphylaxis to red meat. In this study, different alpha-Gal-containing analytes are examined in singleplex and multiplex assays to resolve individual sensitization patterns with IgE against α-Gal...
July 3, 2017: Allergy
https://www.readbyqxmd.com/read/28662189/affective-and-cognitive-behavior-in-the-alpha-galactosidase-a-deficient-mouse-model-of-fabry-disease
#20
Lukas Hofmann, Franziska Karl, Claudia Sommer, Nurcan Üçeyler
Fabry disease is an X-linked inherited lysosomal storage disorder with intracellular accumulation of globotriaosylceramide (Gb3) due to α-galactosidase A (α-Gal A) deficiency. Fabry patients frequently report of anxiety, depression, and impaired cognitive function. We characterized affective and cognitive phenotype of male mice with α-Gal A deficiency (Fabry KO) and compared results with those of age-matched male wildtype (WT) littermates. Young (3 months) and old (≥ 18 months) mice were tested in the naïve state and after i...
2017: PloS One
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