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https://www.readbyqxmd.com/read/29779903/glycosaminoglycans-analysis-in-blood-and-urine-of-patients-with-mucopolysaccharidosis
#1
Shaukat A Khan, Robert W Mason, Roberto Giugliani, Kenji Orii, Toshiyuki Fukao, Yasuyuki Suzuki, Seiji Yamaguchi, Hironori Kobayashi, Tadao Orii, Shunji Tomatsu
To explore the correlation between glycosaminoglycan (GAG) levels and mucopolysaccharidosis (MPS) type, we have evaluated the GAG levels in blood of MPS II, III, IVA, and IVB and urine of MPS IVA, IVB, and VI by tandem mass spectrometry. Dermatan sulfate (DS), heparan sulfate (HS), keratan sulfate (KS; mono-sulfated KS, di-sulfated KS), and the ratio of di-sulfated KS in total KS were measured. Patients with untreated MPS II had higher levels of DS and HS in blood while untreated MPS III had higher levels of HS in blood than age-matched controls...
May 17, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29762133/evidence-and-evolution-of-magnetic-polaron-in-hgcr-sub-2-sub-se-sub-4-sub-investigated-by-electron-spin-resonance
#2
Lailai Li, Li Qin Yan, You-Guo Shi, Peipei Lu, Young Sun
The evidence and evolution of magnetic polarons (MPs) in HgCr<sub>2</sub>Se<sub>4</sub> have been studied by electron spin resonance (ESR), magnetism and conductivity measurements in a temperature range of 5-300 K. A single paramagnetic resonance line is observed in the high-temperature range while multiple resonance lines appear in the low-temperature range. As temperature decreases, the peak-to-peak linewidth ΔHpp shows a minimum at Tmin ≈ 210 K, with the activation energy fitted by small polaron hopping model consistent with the bottleneck mechanism, providing an evidence for existence of small MPs above Tmin...
May 15, 2018: Journal of Physics. Condensed Matter: An Institute of Physics Journal
https://www.readbyqxmd.com/read/29735373/natural-history-of-echocardiographic-abnormalities-in-mucopolysaccharidosis-iii
#3
Carolyn M Wilhelm, Kristen V Truxal, Kim L McBride, John P Kovalchin, Kevin M Flanigan
BACKGROUND: Mucopolysaccharidosis (MPS) type III, Sanfilippo Syndrome, is an autosomal recessive lysosomal storage disorder. MPS I and II patients often develop cardiac involvement leading to early mortality, however there are limited data in MPS III. The objective of this study is to describe cardiac abnormalities in a large group of MPS III patients followed in a longitudinal natural history study designed to determine outcome measures for gene transfer trials. METHODS: A single center study of MPS III patients who were enrolled in the Nationwide Children's Hospital natural history study in 2014...
April 27, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29728280/intensification-of-uv-c-tertiary-treatment-disinfection-and-removal-of-micropollutants-by-sulfate-radical-based-advanced-oxidation-processes
#4
J Rodríguez-Chueca, C García-Cañibano, R-J Lepistö, Á Encinas, J Pellinen, J Marugán
This study explores the enhancement of UV-C tertiary treatment by sulfate radical based Advanced Oxidation Processes (SR-AOPs), including photolytic activation of peroxymonosulfate (PMS) and persulfate (PS) and their photocatalytic activation using Fe(II). Their efficiency was assessed both for the inactivation of microorganisms and the removal or micropollutants (MPs) in real wastewater treatment plant effluents. Under the studied experimental range (UV-C dose 5.7-57 J/L; UV-C contact time 3 to 28 s), the photolysis of PMS and PS (0...
April 21, 2018: Journal of Hazardous Materials
https://www.readbyqxmd.com/read/29707013/the-effect-of-resection-angle-on-stress-distribution-after-root-end-surgery
#5
Jaiane Bandoli Monteiro, Amanda Maria de Oliveira Dal Piva, João Paulo Mendes Tribst, Alexandre Luiz Souto Borges, Rubens Nisie Tango
Introduction: This study aimed to investigate the influence of the resection angle on the stress distribution of retrograde endodontic treated maxillary incisors under oblique-load application. Methods and Materials: A maxillary central incisor which was endodontically treated and restored with a fiber glass post was obtained in a 3-dimensional numerical model and distributed into three groups according to type of resection: control; restored with fiber post without retrograde obturation, R45 and R90 with 45º and 90º resection from tooth axial axis, respectively and restored with Fuji II LC (GC America)...
2018: Iranian Endodontic Journal
https://www.readbyqxmd.com/read/29696349/mucinous-components-assessed-by-magnetic-resonance-imaging-in-primary-rectal-cancer-tissue-before-and-after-chemoradiotherapy-and-tumor-response
#6
Hiroshi Miyakita, Sotaro Sadahiro, Takashi Ogimi, Gota Saito, Kazutake Okada, Akira Tanaka, Toshiyuki Suzuki, Hiroshi Kajiwara, Hiroshi Yamamuro, Takeshi Akiba
BACKGROUND: Mucinous rectal carcinoma has been reported to have a lower survival rate and a poorer histologic response to chemoradiotherapy(CRT). Magnetic resonance imaging (MRI) can accurately evaluate the amount of mucin pools (MP) in primary cancer tissue. We compared the degree of MP on MRI before and after CRT with the histologic findings of resected specimens to investigate the predictors of response to CRT. METHODS: The study group comprised 205 patients with rectal adenocarcinoma who received preoperative CRT...
April 26, 2018: International Journal of Colorectal Disease
https://www.readbyqxmd.com/read/29671225/effectiveness-of-early-hematopoietic-stem-cell-transplantation-in-preventing-neurocognitive-decline-in-mucopolysaccharidosis-type-ii-a-case-series
#7
A Selvanathan, C Ellaway, C Wilson, P Owens, P J Shaw, K Bhattacharya
The early progressive form of the X-linked disorder, Hunter syndrome or mucopolysaccharidosis type II (MPS II) (OMIM #309900), is characterized by cognitive decline, and pulmonary and cardiac complications that often cause death before 20 years of age. Deficiency of the lysosomal enzyme, iduronate-2-sulfatase (EC 3.1.6.13) results in deposition of the glycosaminoglycans, dermatan, and heparan sulfate in various tissues. In recent years, enzyme replacement therapy (ERT) has become the mainstay of treatment, but is expensive and ineffective in arresting cognitive decline...
April 19, 2018: JIMD Reports
https://www.readbyqxmd.com/read/29621675/the-problem-of-overcontrol-perfectionism-emotional-inhibition-and-personality-disorders
#8
Giancarlo Dimaggio, Angus MacBeth, Raffaele Popolo, Giampaolo Salvatore, Filippo Perrini, Aigli Raouna, Cemre Su Osam, Luisa Buonocore, Aldea Bandiera, Antonella Montano
BACKGROUND AND AIMS: Some individuals with Personality Disorders (PD), particularly of a non-Borderline type, present with difficulties relating to over-control of cognitions, emotion and behavior, perfectionistic traits, and impaired social interactions. The current study sought to evaluate the strength of association, and interactions of both emotional inhibition and perfectionism in PD's, after controlling for symptoms and interpersonal problems. METHOD: We recruited a sample of 578 treatment seeking outpatients...
May 2018: Comprehensive Psychiatry
https://www.readbyqxmd.com/read/29606872/porous-se-sio-2-nanocomposites-protect-the-femoral-head-from-methylprednisolone-induced-osteonecrosis
#9
Guoying Deng, Chenyun Dai, Jinyuan Chen, Anqi Ji, Jingpeng Zhao, Yue Zhai, Yingjie Kang, Xijian Liu, Yin Wang, Qiugen Wang
Background: Methylprednisolone (MPS) is an important drug used in therapy of many diseases. However, osteonecrosis of the femoral head is a serious damage in the MPS treatment. Thus, it is imperative to develop new drugs to prevent the serious side effect of MPS. Methods: The potential interferences Se@SiO2 nanocomposites may have to the therapeutic effect of methylprednisolone (MPS) were evaluated by classical therapeutic effect index of acute respiratory distress syndrome (ARDS), such as wet-to-dry weight ratio, inflammatory factors IL-1β and TNF-α...
2018: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/29606503/a-blood-brain-barrier-penetrating-anti-human-transferrin-receptor-antibody-fusion-protein-for-neuronopathic-mucopolysaccharidosis-ii
#10
Hiroyuki Sonoda, Hideto Morimoto, Eiji Yoden, Yuri Koshimura, Masafumi Kinoshita, Galina Golovina, Haruna Takagi, Ryuji Yamamoto, Kohtaro Minami, Akira Mizoguchi, Katsuhiko Tachibana, Tohru Hirato, Kenichi Takahashi
Mucopolysaccharidosis II (MPS II) is an X-linked recessive lysosomal storage disease caused by mutations in the iduronate-2-sulfatase (IDS) gene. Since IDS catalyzes the degradation of glycosaminoglycans (GAGs), deficiency in this enzyme leads to accumulation of GAGs in most cells in all tissues and organs, resulting in severe somatic and neurological disorders. Although enzyme replacement therapy with human IDS (hIDS) has been used for the treatment of MPS II, this therapy is not effective for defects in the CNS mainly because the enzyme cannot cross the blood-brain barrier (BBB)...
March 6, 2018: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/29580682/dose-dependent-prevention-of-metabolic-and-neurologic-disease-in-murine-mps-ii-by-zfn-mediated-in-vivo-genome-editing
#11
Kanut Laoharawee, Russell C DeKelver, Kelly M Podetz-Pedersen, Michelle Rohde, Scott Sproul, Hoang-Oanh Nguyen, Tam Nguyen, Susan J St Martin, Li Ou, Susan Tom, Robert Radeke, Kathleen E Meyer, Michael C Holmes, Chester B Whitley, Thomas Wechsler, R Scott McIvor
Mucopolysaccharidosis type II (MPS II) is an X-linked recessive lysosomal disorder caused by deficiency of iduronate 2-sulfatase (IDS), leading to accumulation of glycosaminoglycans (GAGs) in tissues of affected individuals, progressive disease, and shortened lifespan. Currently available enzyme replacement therapy (ERT) requires lifelong infusions and does not provide neurologic benefit. We utilized a zinc finger nuclease (ZFN)-targeting system to mediate genome editing for insertion of the human IDS (hIDS) coding sequence into a "safe harbor" site, intron 1 of the albumin locus in hepatocytes of an MPS II mouse model...
March 10, 2018: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/29527552/perioperative-airway-management-for-aortic-valve-replacement-in-an-adult-with-mucopolysaccharidosis-type-ii-hunter-syndrome
#12
Kazuchika Suzuki, Hiroaki Sakai, Kenji Takahashi
We herein report anesthetic management during aortic valve replacement for aortic valve regurgitation in a patient with adult mucopolysaccharidosis type II (MPS type 2) (Hunter syndrome). This disorder is rare and related to the accumulation of a mucopolysaccharide in lysosomes. It affects various organs, including the airways, heart, and central nerves. In children with MPS type 2, the risk of airway obstruction during anesthesia/sedation is high, and the degree of difficulty increases with aging. The patient described herein was a 33-year-old male without mental retardation...
2018: JA Clinical Reports
https://www.readbyqxmd.com/read/29520686/can-photobiomodulation-associated-with-implantation-of-mesenchymal-adipose-derived-stem-cells-attenuate-the-expression-of-mmps-and-decrease-degradation-of-type-ii-collagen-in-an-experimental-model-of-osteoarthritis
#13
Tatiane Garcia Stancker, Stella Souza Vieira, Andrey Jorge Serra, Rafael do Nascimento Lima, Regiane Dos Santos Feliciano, José Antônio Silva, Solange Almeida Dos Santos, Marcia Ataize Dos Santos Vieira, Maíra Cecília Brandão Simões, Ernesto Cesar Leal-Junior, Paulo de Tarso Camillo de Carvalho
This study aimed to determine whether photobiomodulation therapy (PBMT) could improve the bioavailability and chondroprotective benefits of mesenchymal stem cells injected into the knees of rats used as an experimental model of osteoarthritis (OA) as well as reduce the expression of matrix metalloproteinases (MMPs) and degradation of type II collagen (COL2-1) in the cartilage. Adipose-derived stem/stromal cells (ADSCs) were collected from three male Fischer 344 rats and characterized by flow cytometry. Fifty female Fischer 344 rats were distributed into five groups of 10 animals each...
March 8, 2018: Lasers in Medical Science
https://www.readbyqxmd.com/read/29397290/complex-care-of-individuals-with-multiple-sulfatase-deficiency-clinical-cases-and-consensus-statement
#14
Rebecca Ahrens-Nicklas, Lars Schlotawa, Andrea Ballabio, Nicola Brunetti-Pierri, Mauricio De Castro, Thomas Dierks, Florian Eichler, Can Ficicioglu, Alan Finglas, Jutta Gaertner, Brian Kirmse, Joerg Klepper, Marcus Lee, Amber Olsen, Giancarlo Parenti, Arastoo Vossough, Adeline Vanderver, Laura A Adang
Multiple sulfatase deficiency (MSD) is an ultra-rare neurodegenerative disorder that results in defective sulfatase post-translational modification. Sulfatases in the body are activated by a unique protein, formylglycine-generating enzyme (FGE) that is encoded by SUMF1. When FGE is absent or insufficient, all 17 known human sulfatases are affected, including the enzymes associated with metachromatic leukodystrophy (MLD), several mucopolysaccharidoses (MPS II, IIIA, IIID, IVA, VI), chondrodysplasia punctata, and X-linked ichthyosis...
March 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29336564/benzophenone-4-promotes-the-growth-of-a-pseudomonas-sp-and-biogenic-oxidation-of-mn-ii
#15
Yangyang Chang, Yaohui Bai, Yang Huo, Jiuhui Qu
Interactions between microbes and micropollutants (MPs) play a crucial role in water purification or treatment. Current studies have generally focused on the direct degradation or cometabolism of MPs. Considering the increasing interest in and importance of the roles of MPs in microbial metabolism, we adopted an Mn(II)-oxidizing Pseudomonas sp. QJX-1 using tyrosine (Tyr) as the sole carbon and nitrogen source to investigate the effects of seven MPs on its growth and function. Six MPs exhibited an inhibition effect on bacterial growth and Mn(II) oxidation...
February 6, 2018: Environmental Science & Technology
https://www.readbyqxmd.com/read/29326871/quantification-of-the-enzyme-activities-of-iduronate-2-sulfatase-n-acetylgalactosamine-6-sulfatase-and-n-acetylgalactosamine-4-sulfatase-using-liquid-chromatography-tandem-mass-spectrometry
#16
Ryuichi Mashima, Mari Ohira, Torayuki Okuyama, Akiya Tatsumi
Mucopolysaccharidosis (MPS) is a genetic disorder characterized by the accumulation of glycosaminoglycans in the body. Of the multiple MPS disease subtypes, several are caused by defects in sulfatases. Specifically, a defect in iduronate-2-sulfatase (ID2S) leads to MPS II, whereas N -acetylgalactosamine-6-sulfatase (GALN) and N -acetylgalactosamine-4-sulfatase (ARSB) defects relate to MPS IVA and MPS VI, respectively. A previous study reported a combined assay for these three disorders in a 96-well plate using a liquid chromatography-tandem mass spectrometry (LC-MS/MS)-based technique (Kumar et al...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29324825/early-and-later-phases-satellite-cell-responses-and-myonuclear-content-with-resistance-training-in-young-men
#17
Felipe Damas, Cleiton A Libardi, Carlos Ugrinowitsch, Felipe C Vechin, Manoel E Lixandrão, Tim Snijders, Joshua P Nederveen, Aline V Bacurau, Patricia Brum, Valmor Tricoli, Hamilton Roschel, Gianni Parise, Stuart M Phillips
Satellite cells (SC) are associated with skeletal muscle remodelling after muscle damage and/or extensive hypertrophy resulting from resistance training (RT). We recently reported that early increases in muscle protein synthesis (MPS) during RT appear to be directed toward muscle damage repair, but MPS contributes to hypertrophy with progressive muscle damage attenuation. However, modulations in acute-chronic SC content with RT during the initial (1st-wk: high damage), early (3rd-wk: attenuated damage), and later (10th-wk: no damage) stages is not well characterized...
2018: PloS One
https://www.readbyqxmd.com/read/29314214/oral-and-craniofacial-manifestations-in-a-hunter-syndrome-patient-with-hematopoietic-stem-cell-transplantation-a-case-report
#18
Rafaela de Oliveira Torres, Andréa Vaz Braga Pintor, Fábio Ribeiro Guedes, Liana Bastos Freitas-Fernandes, Anneliese Lopes Barth, Dafne Dain Gandelman Horovitz, Ivete Pomarico Ribeiro de Souza
We described herein the oral and craniofacial features of a 7-year-old boy, diagnosed in utero with mucopolysaccharidosis II (MPS II), who was treated with hematopoietic stem cell transplantation (HSCT) at 70 days of age. The main oral clinical findings were the following: macroglossia, posterior cross-bite, crowding, pointed cuspid teeth, delayed tooth eruption, retained primary teeth, and enamel hypoplasia. The image examination showed: retention eruption, posterior primary teeth with short roots, absence of some permanent teeth, and stretching of the stylohyoid processes bilaterally...
January 2018: Special Care in Dentistry
https://www.readbyqxmd.com/read/29313030/poly-2-propylacrylic-acid-poly-lactic-co-glycolic-acid-blend-microparticles-as-a-targeted-antigen-delivery-system-to-direct-either-cd4-or-cd8-t-cell-activation
#19
Lirong Yang, Evelyn Bracho-Sanchez, Lawrence P Fernando, Jamal S Lewis, Matthew R Carstens, Craig L Duvall, Benjamin G Keselowsky
Poly(lactic-co-glycolic acid) (PLGA) based microparticles (MPs) are widely investigated for their ability to load a range of molecules with high efficiency, including antigenic proteins, and release them in a controlled manner. Micron-sized PLGA MPs are readily phagocytosed by antigen presenting cells, and localized to endosomes. Due to low pH and digestive enzymes, encapsulated protein cargo is largely degraded and processed in endosomes for MHC-II loading and presentation to CD4+ T cells, with very little antigen delivered into the cytosol, limiting MHC-I antigenic loading and presentation to CD8+ T cells...
June 2017: Bioengineering & Translational Medicine
https://www.readbyqxmd.com/read/29300729/novel-magnetic-multicore-nanoparticles-designed-for-mpi-and-other-biomedical-applications-from-synthesis-to-first-in-vivo-studies
#20
Harald Kratz, Matthias Taupitz, Angela Ariza de Schellenberger, Olaf Kosch, Dietmar Eberbeck, Susanne Wagner, Lutz Trahms, Bernd Hamm, Jörg Schnorr
Synthesis of novel magnetic multicore particles (MCP) in the nano range, involves alkaline precipitation of iron(II) chloride in the presence of atmospheric oxygen. This step yields green rust, which is oxidized to obtain magnetic nanoparticles, which probably consist of a magnetite/maghemite mixed-phase. Final growth and annealing at 90°C in the presence of a large excess of carboxymethyl dextran gives MCP very promising magnetic properties for magnetic particle imaging (MPI), an emerging medical imaging modality, and magnetic resonance imaging (MRI)...
2018: PloS One
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