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https://www.readbyqxmd.com/read/28211716/direct-identification-of-dilute-surface-spins-on-al_-2-o_-3-origin-of-flux-noise-in-quantum-circuits
#1
S E de Graaf, A A Adamyan, T Lindström, D Erts, S E Kubatkin, A Ya Tzalenchuk, A V Danilov
An on-chip electron spin resonance technique is applied to reveal the nature and origin of surface spins on Al_{2}O_{3}. We measure a spin density of 2.2×10^{17}  spins/m^{2}, attributed to physisorbed atomic hydrogen and S=1/2 electron spin states on the surface. This is direct evidence for the nature of spins responsible for flux noise in quantum circuits, which has been an issue of interest for several decades. Our findings open up a new approach to the identification and controlled reduction of paramagnetic sources of noise and decoherence in superconducting quantum devices...
February 3, 2017: Physical Review Letters
https://www.readbyqxmd.com/read/28194615/how-we-manage-adenosine-deaminase-deficient-severe-combined-immune-deficiency-ada-scid
#2
Donald B Kohn, H Bobby Gaspar
Adenosine deaminase-deficient severe combined immune deficiency (ADA SCID) accounts for 10-15% of cases of human SCID. From what was once a uniformly fatal disease, the prognosis for infants with ADA SCID has improved greatly based on the development of multiple therapeutic options, coupled with more frequent early diagnosis due to implementation of newborn screening for SCID. We review the various treatment approaches for ADA SCID including allogeneic hematopoietic stem cell transplantation (HSCT) from a human leukocyte antigen-matched sibling or family member or from a matched unrelated donor or a haplo-identical donor, autologous HSCT with gene correction of the hematopoietic stem cells (gene therapy-GT), and enzyme replacement therapy (ERT) with polyethylene glycol-conjugated adenosine deaminase...
February 14, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28185884/the-emerging-phenotype-of-late-onset-pompe-disease-a-systematic-literature-review
#3
REVIEW
Justin Chan, Ankit K Desai, Zoheb B Kazi, Kaitlyn Corey, Stephanie Austin, Lisa D Hobson-Webb, Laura E Case, Harrison N Jones, Priya S Kishnani
BACKGROUND: Pompe disease is an autosomal recessive disorder caused by deficiency of the lysosomal glycogen-hydrolyzing enzyme acid α-glucosidase (GAA). The adult-onset form, late-onset Pompe disease (LOPD), has been characterized by glycogen accumulation primarily in skeletal, cardiac, and smooth muscles, causing weakness of the proximal limb girdle and respiratory muscles. However, increased scientific study of LOPD continues to enhance understanding of an evolving phenotype. PURPOSE: To expand our understanding of the evolving phenotype of LOPD since the approval of enzyme replacement therapy (ERT) with alglucosidase alfa (Myozyme™/Lumizyme™) in 2006...
December 11, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28185830/children-with-type-1-gaucher-disease-changing-profiles-in-the-21st-century
#4
Deborah Elstein, Gheona Altarescu, Aya Abrahamov, Ari Zimran
Gaucher disease (GD) has phenotypic variability. Increased GD awareness especially among at-risk Ashkenazi Jews (AJ) and availability of non-invasive diagnosis induced trend to prenatal screening. We retrospectively assessed pediatric (<16years) Israeli AJ GD patients to ascertain demographics and phenotype at presentation and over-time because many were identified by large-scale screening. 55/67 patients born since 01/01/2000 are AJ with non-neuronopathic GD: 28 (50.9%) are N370S/N370S; 24 (43.6%) are N370S/other; 3 (3...
December 19, 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28162835/contribution-of-hydrochemical-and-geoelectrical-approaches-to-investigate-salinization-process-and-seawater-intrusion-in-the-coastal-aquifers-of-chaouia-morocco
#5
Saliha Najib, Ahmed Fadili, Khalid Mehdi, Joëlle Riss, Abdelhadi Makan
This study aims to identify groundwater salinization origin and to determine seawater intrusion extension toward the inland in Chaouia, Morocco. To reach these objectives, firstly, 46 groundwater samples were analyzed for major chemical elements during January 2012 and, secondly, 10 electrical resistivity tomography (ERT) profiles were performed perpendicularly to the coastal fringe. Statistical analysis provided the distinction between three Clusters reflecting different hydrochemical processes. Cluster I and Cluster II-a showed a high water electrical conductivity (EC) (from 2...
January 31, 2017: Journal of Contaminant Hydrology
https://www.readbyqxmd.com/read/28161408/anti-blys-antibody-reduces-the-immune-reaction-against-enzyme-and-enhances-the-efficacy-of-enzyme-replacement-therapy-in-fabry-disease-model-mice
#6
Yohei Sato, Hiroyuki Ida, Toya Ohashi
Formation of antibodies against a therapeutic enzyme is an important complication during enzyme replacement therapy (ERT) for lysosomal storage diseases. Fabry disease (FD) is caused by a deficiency of alpha-galactosidase (GLA), which results in the accumulation of globotriaosylceramide (GL-3). We have shown immune tolerance induction (ITI) during ERT in FD model mice by using an anti-B lymphocyte stimulator (anti-BlyS) antibody (belimumab). A single dose of the anti-BlyS antibody temporarily lowered the percentage of B cells and IgG antibody titer against recombinant human GLA...
February 2, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28160363/establishment-of-a-tobacco-by2-cell-line-devoid-of-plant-specific-xylose-and-fucose-as-a-platform-for-the-production-of-biotherapeutic-proteins
#7
Uri Hanania, Tami Ariel, Yoram Tekoah, Liat Fux, Maor Sheva, Yehuda Gubbay, Mara Weiss, Dina Oz, Yaniv Azulay, Albina Turbovski, Yehava Forster, Yoseph Shaaltiel
Plant produced glycoproteins contain N-linked glycans with plant-specific residues of β(1,2)-xylose and core α(1,3)-fucose, which do not exist in mammalian derived proteins. Although our experience with two enzymes that are used for enzyme replacement therapy (ERT) does not indicate that the plant sugar residues have deleterious effects, we made a conscious decision to eliminate these moieties from plant-expressed proteins. We knocked out the β(1,2)-xylosyltranferase (XylT) and the α(1,3)-fucosyltransferase (FucT) genes, using CRISPR/Cas9 genome editing, in Nicotiana...
February 3, 2017: Plant Biotechnology Journal
https://www.readbyqxmd.com/read/28154884/antibody-mediated-enzyme-replacement-therapy-targeting-both-lysosomal-and-cytoplasmic-glycogen-in-pompe-disease
#8
Haiqing Yi, Tao Sun, Dustin Armstrong, Scott Borneman, Chunyu Yang, Stephanie Austin, Priya S Kishnani, Baodong Sun
: Pompe disease is characterized by accumulation of both lysosomal and cytoplasmic glycogen primarily in skeletal and cardiac muscles. Mannose-6-phosphate receptor-mediated enzyme replacement therapy (ERT) with recombinant human acid α-glucosidase (rhGAA) targets the enzyme to lysosomes and thus is unable to digest cytoplasmic glycogen. Studies have shown that anti-DNA antibody 3E10 penetrates living cells and delivers "cargo" proteins to the cytosol or nucleus via equilibrative nucleoside transporter ENT2...
February 2, 2017: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/28124022/electrical-resistivity-tomography-data-across-the-hockai-fault-zone-ardenne-belgium
#9
Thomas Lecocq, Thierry Camelbeeck
In this work, we present the result of a large-scale geophysical survey that had the objective of identifying the subsurface characteristics and the NE-SW extension of the Hockai Fault Zone: a major NNW-SSE oriented crustal-rooted fault zone crossing the Stavelot-Venn Massif (Eastern Belgium). 31 two-dimensional electrical resistivity tomography (ERT) profiles are presented, resulting in 10,679 m of 2D sections. All profiles were acquired between 2008 and 2010 using a single channel ABEM Terrameter SAS1000 instrument connected to a 64 electrodes setup of maximum 315 m extent which was often extended using the roll-along technique...
April 2017: Data in Brief
https://www.readbyqxmd.com/read/28119823/pilot-study-of-the-safety-and-effect-of-adalimumab-on-pain-physical-function-and-musculoskeletal-disease-in-mucopolysaccharidosis-types-i-and-ii
#10
Lynda E Polgreen, Alicia Kunin-Batson, Kyle Rudser, Richard K Vehe, Jeanine J Utz, Chester B Whitley, Patricia Dickson
Mucopolysaccharidosis I and II are lysosomal storage disorders that, despite treatment with hematopoietic cell transplantation (HCT) and/or enzyme replacement therapy (ERT), continue to cause significant skeletal abnormalities leading to pain, stiffness, physical dysfunction, and short stature. Tumor necrosis factor - alpha (TNF-α) is elevated in individuals with MPS I and II and associated with pain and physical dysfunction. Therefore, we evaluated the safety and effects of the TNF-α inhibitor adalimumab in patients with MPS I and II in a 32-week, randomized, double blind, placebo-controlled, crossover study of adalimumab at a dose of 20 mg (weight 15-<30 kg) or 40 mg (weight ≥ 30 kg) administered subcutaneously every other week or saline placebo for 16 weeks...
March 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28119821/immune-tolerance-induction-for-laronidase-treatment-in-mucopolysaccharidosis-i
#11
Roberto Giugliani, Taiane Alves Vieira, Clarissa Gutierrez Carvalho, Maria-Veronica Muñoz-Rojas, Alla N Semyachkina, Victoria Y Voinova, Susan Richards, Gerald F Cox, Yong Xue
: Enzyme replacement therapy (ERT) can produce anti-drug antibody (ADA) responses that reduce efficacy or lead to hypersensitivity reactions. Six patients with severe mucopolysaccharidosis type I (MPS I/Hurler syndrome) who did not receive hematopoietic stem cell transplantation underwent an immunosuppression regimen prior to initiating ERT with laronidase. The primary endpoint for immune tolerance induction was the number of patients with an ADA titer ≤ 3200 after 24 weeks of laronidase at the labeled dose...
March 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28114109/methods-for-calculating-the-electrode-position-jacobian-for-impedance-imaging
#12
Alistair Boyle, Michael Crabb, Markus Jehl, William Lionheart, Andy Adler
Electrical Impedance Tomography (EIT) or Electrical Resistivity Tomography (ERT) apply current and measure voltages at the boundary of a domain through electrodes. The movement or incorrect placement of electrodes may lead to modelling errors that result in significant reconstructed image artifacts. These errors may be accounted for by allowing for electrode position estimates in the model. Movement may be reconstructed through a first-order approximation, the electrode position Jacobian. A reconstruction that incorporates electrode position estimates and conductivity can significantly reduce image artifacts...
January 23, 2017: Physiological Measurement
https://www.readbyqxmd.com/read/28078498/perinatal-exposure-to-bisphenol-a-or-diethylstilbestrol-increases-the-susceptibility-to-develop-mammary-gland-lesions-after-estrogen-replacement-therapy-in-middle-aged-rats
#13
Ayelen L Gomez, Melisa B Delconte, Gabriela A Altamirano, Lucia Vigezzi, Veronica L Bosquiazzo, Luís F Barbisan, Jorge G Ramos, Enrique H Luque, Mónica Muñoz-de-Toro, Laura Kass
The development of the mammary gland is a hormone-regulated event. Several factors can dysregulate its growth and make the gland more susceptible to cellular transformation. Among these factors, perinatal exposure to xenoestrogens and hormone replacement therapy has been associated with increased risk of developing breast cancer. Here, we assessed the effects induced by estrogen replacement therapy (ERT) in ovariectomized (OVX) middle-aged rats and whether perinatal exposure to diethylstilbestrol (DES) or bisphenol A (BPA) modified these effects in the mammary gland...
January 11, 2017: Hormones & Cancer
https://www.readbyqxmd.com/read/28075357/correlations-between-endomyocardial-biopsies-and-cardiac-manifestations-in-taiwanese-patients-with-the-chinese-hotspot-ivs4-919g-a-mutation-data-from-the-fabry-outcome-survey
#14
Ting-Rong Hsu, Fu-Pang Chang, Tzu-Hung Chu, Shih-Hsien Sung, Svetlana Bizjajeva, Wen-Chung Yu, Dau-Ming Niu
We retrospectively evaluated correlations between cardiac manifestations and globotriaosylceramide (Gb3) accumulation in cardiomyocytes from Taiwanese patients with Fabry disease and the IVS4+919G>A (IVS4) mutation who underwent endomyocardial biopsy (Shire; Fabry Outcome Survey data; extracted January 2015). Of 24 males and six females (median age [Q1; Q3] at biopsy 60.4 [57.4; 64.1] and 61.3 [60.4; 65.1] years, respectively), 13 males (54.2%) and five females (83.3%) received agalsidase alfa enzyme replacement therapy (ERT) before biopsy...
January 9, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28070711/immune-suppression-during-preclinical-drug-development-mitigates-immunogenicity-mediated-impact-on-therapeutic-exposure
#15
Jonathan Herskovitz, Josiah Ryman, Theingi Thway, Stephanie Lee, Lei Zhou, Narendra Chirmule, Bernd Meibohm, Vibha Jawa
In the clinical setting, anti-drug antibodies (ADA) against biotherapeutics can influence patient safety and interfere with product efficacy. High immunogenicity has been addressed in clinic by concomitant immune suppression, such as co-administration of methotrexate with enzyme replacement therapy (ERT) and combination tacrolimus/sirolimus treatment for prophylaxis against organ transplant rejection. This study investigates the use of such immune suppressants in mitigating ADA responses to a fully human monoclonal antibody (mAb1) in preclinical animal studies...
January 9, 2017: AAPS Journal
https://www.readbyqxmd.com/read/28065440/glycosaminoglycan-levels-in-dried-blood-spots-of-patients-with-mucopolysaccharidoses-and-mucolipidoses
#16
Francyne Kubaski, Yasuyuki Suzuki, Kenji Orii, Roberto Giugliani, Heather J Church, Robert W Mason, Vũ Chí Dũng, Can Thi Bich Ngoc, Seiji Yamaguchi, Hironori Kobayashi, Katta M Girisha, Toshiyuki Fukao, Tadao Orii, Shunji Tomatsu
: Mucopolysaccharidoses (MPSs) and mucolipidoses (ML) are groups of lysosomal storage disorders in which lysosomal hydrolases are deficient leading to accumulation of undegraded glycosaminoglycans (GAGs), throughout the body, subsequently resulting in progressive damage to multiple tissues and organs. Assays using tandem mass spectrometry (MS/MS) have been established to measure GAGs in serum or plasma from MPS and ML patients, but few studies were performed to determine whether these assays are sufficiently robust to measure GAG levels in dried blood spots (DBS) of patients with MPS and ML...
December 22, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28053586/comparison-of-phenotypic-and-pcr-methods-for-detection-of-carbapenemases-production-by-enterobacteriaceae
#17
Maryam AlTamimi, Ali AlSalamah, Manal AlKhulaifi, Hisham AlAjlan
Dissemination of carbapenem resistance via Enterobacteriaceae, particularly among Klebsiella pneumoniae and Escherichia coli, is a major public health concern. Rapid methods for determining antimicrobial susceptibility are important to ensure adequate and appropriate use of antimicrobial agents and to limit the spread of these bacteria. In the current study, we compared the rapidity, sensitivity and specificity of traditional methods and molecular-based Xpert Carba-R PCR assay to identify sixty isolates, (26 E...
January 2017: Saudi Journal of Biological Sciences
https://www.readbyqxmd.com/read/28050459/cardiovascular-abnormalities-in-egyptian-children-with-mucopolysaccharidoses
#18
Laila Selim, Nehal Abdelhamid, Emad Salama, Amera Elbadawy, Iman Gamaleldin, Mohamed Abdelmoneim, Abeer Selim
INTRODUCTION: The Mucopolysaccharidoses (MPS) are rare inherited metabolic disorders. They are characterized by the progressive systemic deposition of Glycosaminoglycans (GAGs). GAGs accumulate in the myocardium and the cardiac valves. Enzyme Replacement Therapy (ERT) is available for MPS I, II, and VI. However, ERT does not appear to improve cardiac valve disease in patients with valve disease present at the start of ERT. AIM: To evaluate the cardiac involvement in Egyptian children with MPS...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28040394/long-term-hematological-visceral-and-growth-outcomes-in-children-with-gaucher-disease-type-3-treated-with-imiglucerase-in-the-international-collaborative-gaucher-group-gaucher-registry
#19
Amal El-Beshlawy, Anna Tylki-Szymanska, Ashok Vellodi, Nadia Belmatoug, Gregory A Grabowski, Edwin H Kolodny, Julie L Batista, Gerald F Cox, Pramod K Mistry
In Gaucher disease (GD), deficiency of lysosomal acid β-glucosidase results in a broad phenotypic spectrum that is classified into three types based on the absence (type 1 [GD1]) or presence and severity of primary central nervous system involvement (type 2 [GD2], the fulminant neuronopathic form, and type 3 [GD3], the milder chronic neuronopathic form). Enzyme replacement therapy (ERT) with imiglucerase ameliorates and prevents hematological and visceral manifestations in GD1, but data in GD3 are limited to small, single-center series...
December 6, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28029576/could-enzyme-replacement-therapy-promote-immune-tolerance-in-gaucher-disease-type-1
#20
Marina Cadena Matta, Filippo Vairo, Leuridan Cavalcante Torres, Ida Schwartz
Among the lysosomal storage disorders, Gaucher disease (GD) features some of the most striking alterations in the immune system, including increased levels of cytokines and chemokines. Although studies have demonstrated the efficacy of enzyme replacement therapy (ERT) for GD, the ideal dosage remains controversial. In this study, we report differences in levels of cytokines (IL-6, TNF-a, and IFN-y) and chemokines (IL-8, IP-10, and MCP-1) in patients with GD type 1 treated with different ERT dosages and treatment durations...
October 24, 2016: Blood Cells, Molecules & Diseases
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