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Mps I, mps

Srikant Rangaraju, Syed Ali Raza, Noel Xiang'An Li, Ranjita Betarbet, Eric B Dammer, Duc Duong, James J Lah, Nicholas T Seyfried, Allan I Levey
In the central nervous system (CNS), microglia are innate immune mononuclear phagocytes (CNS MPs) that can phagocytose infectious particles, apoptotic cells, neurons, and pathological protein aggregates, such as Aβ in Alzheimer's disease (AD). While CD11b+ CD45low microglia account for the majority of CNS MPs, a small population of CD11b+ CD45high CNS MPs is also recognized in AD that surround Aβ plaques. These transcriptionally and pathologically unique CD45high cells have unclear origin and undefined phagocytic characteristics...
2018: Frontiers in Immunology
Ande Rostvall, Wen Zhang, Wiebke Dürig, Gunno Renman, Karin Wiberg, Lutz Ahrens, Pablo Gago-Ferrero
This study evaluated the performance of five different sorbents (granular activated carbon (GAC), GAC + Polonite® (GAC + P), Xylit, lignite and sand) for a set of 83 micropollutants (MPs) (pharmaceuticals, perfluoroalkyl substances (PFASs), personal care products, artificial sweeteners, parabens, pesticide, stimulants), together representing a wide range of physicochemical properties. Treatment with GAC and GAC + P provided the highest removal efficiencies, with average values above 97%. Removal rates were generally lower for Xylit (on average 74%) and lignite (on average 68%), although they proved to be highly efficient for a few individual MPs...
March 6, 2018: Water Research
Julie B Eisengart, Kyle D Rudser, Yong Xue, Paul Orchard, Weston Miller, Troy Lund, Ans Van der Ploeg, Jean Mercer, Simon Jones, Karl Eugen Mengel, Seyfullah Gökce, Nathalie Guffon, Roberto Giugliani, Carolina F M de Souza, Elsa G Shapiro, Chester B Whitley
PurposeEarly treatment is critical for mucopolysaccharidosis type I (MPS I), justifying its incorporation into newborn screening. Enzyme replacement therapy (ERT) treats MPS I, yet presumptions that ERT cannot penetrate the blood-brain barrier (BBB) support recommendations that hematopoietic cell transplantation (HCT) treat the severe, neurodegenerative form (Hurler syndrome). Ethics precludes randomized comparison of ERT with HCT, but insight into this comparison is presented with an international cohort of patients with Hurler syndrome who received long-term ERT from a young age...
March 8, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Benoit Stijlemans, Patrick De Baetselier, Stefan Magez, Jo A Van Ginderachter, Carl De Trez
African trypanosomosis (AT) is a chronically debilitating parasitic disease of medical and economic importance for the development of sub-Saharan Africa. The trypanosomes that cause this disease are extracellular protozoan parasites that have developed efficient immune escape mechanisms to manipulate the entire host immune response to allow parasite survival and transmission. During the early stage of infection, a profound pro-inflammatory type 1 activation of the mononuclear phagocyte system (MPS), involving classically activated macrophages (i...
2018: Frontiers in Immunology
Birendra Chaurasiya, Liping Huang, Yunai Du, Baoqiang Tang, Zijie Qiu, Li Zhou, Jiasheng Tu, Chunmeng Sun
In this study, we prepared paclitaxel (PTX) loaded bovine serum albumin (BSA) microparticles (MPs) of different sizes (0.5, 1.0, and 3.0 µm) and converted them into dry powders (DPs) of a uniform size (∼5.0 µm) through spray-drying techniques. The aim of preparing different sized PTX-MPs is to investigate the size-based in vivo biodistribution and retention of PTX in the lungs after intratracheal administration. Following the in vitro characterizations, the anti-tumor efficacy of the DPs containing differently sized PTX-BSA-MPs administered through intratracheal insufflation was compared with intravenously administered PTX solution (Taxol)...
February 26, 2018: International Journal of Pharmaceutics
Elina Makino, Helen Klodnitsky, John Leonard, James Lillie, Troy C Lund, John Marshall, Jennifer Nietupski, Paul J Orchard, Weston P Miller, Clifford Phaneuf, Drew Tietz, Mariet L Varban, Marissa Donovan, Alexey Belenki
Certain recessively inherited diseases result from an enzyme deficiency within lysosomes. In mucopolysaccharidoses (MPS), a defect in glycosaminoglycan (GAG) degradation leads to GAG accumulation followed by progressive organ and multiple system dysfunctions. Current methods of GAG analysis used to diagnose and monitor the diseases lack sensitivity and throughput. Here we report a LC-MS method with accurate metabolite mass analysis for identifying and quantifying biomarkers for MPS type I without the need for extensive sample preparation...
February 27, 2018: Scientific Reports
Heather G Mack, R C Andrew Symons, Gerard de Jong
Purpose: To report retinal findings in two patients with mucopolysaccharidosis type I (MPS I) receiving human recombinant alpha-l-iduronidase (Laronidase) as enzyme replacement therapy. Observations: Patient 1 had visual acuity 20/20 right eye, 20/25 left eye and unremarkable anterior segment and retinal examination. Optical coherence tomography (OCT) scanning demonstrated parafoveal thinning and subfoveal hyperreflectant material. Patient 2 had visual acuity 20/20 both eyes, with dense nuclear cataract both eyes...
March 2018: American Journal of Ophthalmology Case Reports
Sunil H Koli, Bhavana V Mohite, Rahul K Suryawanshi, Hemant P Borase, Satish V Patil
The development of a safe and eco-friendly method for metal nanoparticle synthesis has an increasing demand, due to emerging environmental and biological harms of hazardous chemicals used in existing nanosynthesis methods. The present investigation reports a rapid one-step, eco-friendly and green approach for the formation of nanosized silver particles (AgNPs) using extracellular non-toxic-colored fungal metabolites (Monascus pigments-MPs). The formation of nanosized silver particles utilizing Monascus pigments was confirmed after exposure of reaction mixture to sunlight, by visually color change and further established by spectrophotometric analysis...
February 10, 2018: Bioprocess and Biosystems Engineering
Tanner Stokes, Amy J Hector, Robert W Morton, Chris McGlory, Stuart M Phillips
Skeletal muscle supports locomotion and serves as the largest site of postprandial glucose disposal; thus it is a critical organ for physical and metabolic health. Skeletal muscle mass is regulated by the processes of muscle protein synthesis (MPS) and muscle protein breakdown (MPB), both of which are sensitive to external loading and aminoacidemia. Hyperaminoacidemia results in a robust but transient increase in rates of MPS and a mild suppression of MPB. Resistance exercise potentiates the aminoacidemia-induced rise in MPS that, when repeated over time, results in gradual radial growth of skeletal muscle (i...
February 7, 2018: Nutrients
Marat I Kamalov, Trinh Đặng, Natalia V Petrova, Alexander V Laikov, Duong Luong, Rezeda A Akhmadishina, Andrei N Lukashkin, Timur I Abdullin
A new self-assembled formulation of methylprednisolone succinate (MPS) based on a carboxylated trifunctional block copolymer of ethylene oxide and propylene oxide (TBC-COOH) was developed. TBC-COOH and MPS associated spontaneously at increased concentrations in aqueous solutions to form almost monodisperse mixed micelles (TBC-COOH/MPS) with a hydrodynamic diameter of 19.6 nm, zeta potential of -27.8 mV and optimal weight ratio ∼1:6.3. Conditions for the effective formation of TBC-COOH/MPS were elucidated by comparing copolymers and glucocorticoids with different structure...
January 11, 2018: Colloids and Surfaces. B, Biointerfaces
Dani L Webber, Amanda Choo, Laura J Hewson, Paul J Trim, Marten F Snel, John J Hopwood, Robert I Richards, Kim M Hemsley, Louise V O'Keefe
Mucopolysaccharidosis type IIIA (MPS IIIA) is a lysosomal storage disorder resulting from the deficit of the N-sulfoglucosamine sulfohydrolase (SGSH) enzyme that leads to accumulation of partially-degraded heparan sulfate. MPS IIIA is characterized by severe neurological symptoms, clinically presenting as Sanfilippo syndrome, for which no effective therapy is available. The lysosomal SGSH enzyme is conserved in Drosophila and we have identified increased levels of heparan sulfate in flies with ubiquitous knockdown of SGSH/CG14291...
February 1, 2018: Experimental Neurology
E Poletto, G Pasqualim, R Giugliani, U Matte, G Baldo
Mucopolysaccharidosis type I (MPS I) is a rare disorder caused by deleterious sequence variants in the α-L-iduronidase (IDUA) gene. More than 200 pathogenic variants have been described so far, but their frequencies have not yet been analysed on a worldwide scale. To address this, we analysed the genotypes of MPS I patients from thirty-five published studies papers. The most common pathogenic variant observed was p.Trp402Ter. With frequencies of up to 63%, it was the major allele in most European countries, America and Australia...
February 2, 2018: Clinical Genetics
Theodore Pipikos, Alkistis Kapelouzou, Diamantis I Tsilimigras, Yannis Fostinis, Marina Pipikou, Athanassios Theodorakos, Antonis N Pavlidis, Christos Kontogiannis, Dennis V Cokkinos, Maria Koutelou
BACKGROUND: Acute myocardial infarction (AMI) is considered a major cause of death and disability. Myocardial perfusion scintigraphy (MPS) as a non-invasive diagnostic imaging procedure and certain biomarkers associated with myocardial ischemia (ISCH), such as ischemia-modified albumin (IMA), neuropeptide Y (NPY), N-terminal pro b-type natriuretic peptide (NT-proBNP), and high-sensitivity troponin T (hsTnT) could probably aid in the detection of myocardial infarction. METHODS: Between December 2011 and June 2012, we prospectively analyzed patients who underwent a MPS study with the clinical question of myocardial ISCH...
January 29, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
Rucha Patil, Smrati Bajpai, Kanjaksha Ghosh, Shrimati Shetty
Promising biomarkers which may help predict the risk of developing severe dengue virus infection (DVI) are lacking and will be helpful. Thus the main aim of this study was to analyze the role of cell-derived microparticles (MP) in DVI. Sixty patients with DVI i.e. 18: dengue with warning signs (DWS); 1: DSS and 41: dengue without warning signs (DWOS); along with 15 controls (other febrile illness) were included in the study. The following MPs were assessed: annexinV, platelet (CD41a), red blood cell (RBC) (CD235a) and activated endothelial (CD62e) MPs...
January 26, 2018: Acta Tropica
Esteban Alberto Gonzalez, Giselle Renata Martins, Angela Maria Vicente Tavares, Michelle Viegas, Edina Poletto, Roberto Giugliani, Ursula Matte, Guilherme Baldo
Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder with multisystemic features, including heart enlargement, heart valve dysfunction, and aortic stiffness and dilatation. Previous studies have shown that MPS I mice overexpress cathepsin B (CtsB) in multiple tissues, including those from the cardiovascular system. Here, we hypothesized that inhibition of CtsB could ameliorate cardiac function parameters, as well as aorta and valve abnormalities found in MPS I. First, we found that total elastase activity in an MPS I aorta is elevated...
January 20, 2018: Life Sciences
Takanori Konishi, Rebecca M Schuster, Alex B Lentsch
Yes-associated protein (YAP) and transcriptional co-activator with PDZ-binding motif (TAZ) are key regulators of cell proliferation and organ size, however, their physiological contribution after liver injury has not been fully understood. In this study, we sought to determine the role of YAP and TAZ during liver recovery after ischemia-reperfusion (I/R). A murine model of partial (70%) I/R was used to induce liver injury and study the reparative and regenerative response. After liver injury, there was marked activation and proliferation of hepatic stellate cells...
January 11, 2018: American Journal of Physiology. Gastrointestinal and Liver Physiology
Göran Nilsson, Jeffrey R Budd, Neil Greenberg, Vincent Delatour, Robert Rej, Mauro Panteghini, Ferruccio Ceriotti, Heinz Schimmel, Cas Weykamp, Thomas Keller, Johanna E Camara, Chris Burns, Hubert W Vesper, Finlay MacKenzie, W Greg Miller
A process is described to assess the commutability of a reference material (RM) intended for use as a calibrator, trueness control, or external quality assessment sample based on the difference in bias between an RM and clinical samples (CSs) measured using 2 different measurement procedures (MPs). This difference in bias is compared with a criterion based on a medically relevant difference between an RM and CS results to make a conclusion regarding commutability. When >2 MPs are included, the commutability is assessed pairwise for all combinations of 2 MPs...
January 18, 2018: Clinical Chemistry
Miguel Sampayo-Cordero, Bernat Miguel-Huguet, Almudena Pardo-Mateos, Marc Moltó-Abad, Cecilia Muñoz-Delgado, Jordi Pérez-López
BACKGROUND: Case reports might have a prominent role in the rare diseases field, due to the small number of patients affected by one such disease. A previous systematic review regarding the efficacy of laronidase therapy in patients with mucopolysaccharidosis type I (MPS-I) who initiated enzyme replacement therapy (ERT) in adult age has been published. The review included a meta-analysis of 19 clinical studies and the description of eleven case reports. It was of interest to perform a meta-analysis of those case reports to explore the role of such meta-analyses as a tool for evidence-based medicine in rare diseases...
January 5, 2018: Molecular Genetics and Metabolism
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 co-metabolism of MPs. Considering the increasing interest in and importance of the roles of MPs in microbial metabolism, we adopted a 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...
January 16, 2018: Environmental Science & Technology
M R Oliveira, I Schwartz, L S Costa, H Maia, M Ribeiro, L B Guerreiro, A Acosta, N S Rocha
OBJECTIVE: To describe the perceptions of patients, their caregivers, and their healthcare providers to the development of a new specific instrument for assessment of the quality of life (QoL) in patients with mucopolysaccharidoses (MPS) using a qualitative focus group (FG) design. FGs were held in two Brazilian states (Rio Grande do Sul and Rio de Janeiro). RESULTS: Three versions of the new instrument were developed, each for a different age group: children (age 8-12 years), adolescents (age 13-17), and adults (age ≥ 18)...
January 15, 2018: BMC Research Notes
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