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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
#1
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/29751845/beneath-the-floor-re-analysis-of-neurodevelopmental-outcomes-in-untreated-hurler-syndrome
#2
Elsa G Shapiro, Chester B Whitley, Julie B Eisengart
BACKGROUND: Hurler syndrome (MPS IH), the severe, neurodegenerative form of type one mucopolysaccharidosis, is associated with rapid neurocognitive decline during toddlerhood and multi-system dysfunction. It is now standardly treated with hematopoietic cell transplantation (HCT), which halts accumulating disease pathology and prevents early death. While norm-based data on developmental functioning in untreated children have previously demonstrated neurocognitive decline, advances in methodology for understanding the cognitive functioning of children with neurodegenerative diseases have highlighted that the previous choice of scores to report results was not ideal...
May 11, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29748879/poly-l-ornithine-fucoidan-coated-calcium-carbonate-microparticles-by-layer-by-layer-self-assembly-technique-for-cancer-theranostics
#3
Pei Wang, Ranjith Kumar Kankala, Jingqian Fan, Ruimin Long, Yuangang Liu, Shibin Wang
Recently, the layer-by-layer (LbL) self-assembly technology has attracted the enormous interest of researchers in synthesizing various pharmaceutical dosage forms. Herewith, we designed a biocompatible drug delivery system containing the calcium carbonate microparticles (CaCO3 MPs) that coated with the alternatively charged polyelectrolytes, i.e., poly-L-ornithine (PLO)/fucoidan by LbL self-assembly process (LbL MPs). Upon coating with the polyelectrolytes, the mean particle size of MPs obtained from SEM observations increased from 1...
May 10, 2018: Journal of Materials Science. Materials in Medicine
https://www.readbyqxmd.com/read/29735373/natural-history-of-echocardiographic-abnormalities-in-mucopolysaccharidosis-iii
#4
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/29705972/p-tau-and-subunit-c-mitochondrial-atp-synthase-accumulation-in-the-central-nervous-system-of-a-woman-with-hurler-scheie-syndrome-treated-with-enzyme-replacement-therapy-for-12-years
#5
Hiroshi Kobayashi, Masamichi Ariga, Yohei Sato, Masako Fujiwara, Nei Fukasawa, Takahiro Fukuda, Hiroyuki Takahashi, Masahiro Ikegami, Motomichi Kosuga, Torayuki Okuyama, Yoshikatsu Eto, Hiroyuki Ida
We report an autopsy case of a woman with mucopolysaccharidosis type I (MPS I) Hurler-Scheie syndrome who was treated with enzyme replacement therapy (ERT) for 12 years. This was the first case of MPS I treated with ERT in Japan. Pathological analysis showed no glycosaminoglycan accumulation in the liver and spleen as a result of long-term ERT, although severe aortic stenosis, diffuse intimal hyperplasia of the coronary artery, and fibrous hypertrophy of the endocardium were observed. Additionally, we detected subunit c mitochondrial ATP synthase (SCMAS) accumulation and mild tauopathy (hyperphosphorylated tau or p-tau, both 3-repeat and 4-repeat tau accumulation) in the same area of the cerebral limbic system and central gray matter of the mid brain and pons...
April 29, 2018: JIMD Reports
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/29683519/children-with-mucopolysaccharidosis-risk-progressive-visual-dysfunction-despite-haematopoietic-stem-cell-transplants
#7
Kristina Teär Fahnehjelm, Monica Olsson, Enping Chen, Jürg Hengstler, Karin Naess, Jacek Winiarski
AIM: This prospective study assessed the long-term ocular and visual outcomes of children with mucopolysaccharidoses type I Hurler syndrome (MPS IH) who were treated with haematopoietic stem cell transplants (HSCT). METHODS: Clinical ophthalmological assessments were performed on eight patients at the St Erik Eye Hospital, Stockholm, Sweden, from 2001-2018: The median age at diagnosis and HSCT were 12.2 (range 5.0-16.4) and 16.7 (8.0-20.4) months. The last eye examination was at a median of 13...
April 23, 2018: Acta Paediatrica
https://www.readbyqxmd.com/read/29679929/a-phylogenetic-framework-facilitates-y-str-variant-discovery-and-classification-via-massively-parallel-sequencing
#8
Tunde I Huszar, Mark A Jobling, Jon H Wetton
Short tandem repeats on the male-specific region of the Y chromosome (Y-STRs) are permanently linked as haplotypes, and therefore Y-STR sequence diversity can be considered within the robust framework of a phylogeny of haplogroups defined by single nucleotide polymorphisms (SNPs). Here we use massively parallel sequencing (MPS) to analyse the 23 Y-STRs in Promega's prototype PowerSeq™ Auto/Mito/Y System kit (containing the markers of the PowerPlex® Y23 [PPY23] System) in a set of 100 diverse Y chromosomes whose phylogenetic relationships are known from previous megabase-scale resequencing...
April 12, 2018: Forensic Science International. Genetics
https://www.readbyqxmd.com/read/29677694/3s-systematic-systemic-and-systems-biology-and-toxicology
#9
Lena Smirnova, Nicole Kleinstreuer, Raffaella Corvi, Andre Levchenko, Suzanne C Fitzpatrick, Thomas Hartung
A biological system is more than the sum of its parts - it accomplishes many functions via synergy. Deconstructing the system down to the molecular mechanism level necessitates the complement of reconstructing functions on all levels, i.e., in our conceptualization of biology and its perturbations, our experimental models and computer modelling. Toxicology contains the somewhat arbitrary subclass "systemic toxicities"; however, there is no relevant toxic insult or general disease that is not systemic...
2018: ALTEX
https://www.readbyqxmd.com/read/29662787/steroid-associated-osteonecrosis-animal-model-in-rats
#10
Li-Zhen Zheng, Jia-Li Wang, Ling Kong, Le Huang, Li Tian, Qian-Qian Pang, Xin-Luan Wang, Ling Qin
Objective: Established preclinical disease models are essential for not only studying aetiology and/or pathophysiology of the relevant diseases but more importantly also for testing prevention and/or treatment concept(s). The present study proposed and established a detailed induction and assessment protocol for a unique and cost-effective preclinical steroid-associated osteonecrosis (SAON) in rats with pulsed injections of lipopolysaccharide (LPS) and methylprednisolone (MPS). Methods: Sixteen 24-week-old male Sprague-Dawley rats were used to induce SAON by one intravenous injection of LPS (0...
April 2018: Journal of Orthopaedic Translation
https://www.readbyqxmd.com/read/29649608/standalone-cranio-cervical-decompression-is-feasible-in-children-with-mucopolysaccharidosis-type-i-iva-and-vi
#11
Harald Krenzlin, Ta-Chih Tan, Christina Lampe, Christian Lampe, Markus Knuf, Peter Horn, Manfred Schwarz
BACKGROUND CONTEXT: In patients with Mucopolysaccharidosis (MPS), glycosaminoglycans deposits in the dura mater and supporting ligaments cause spinal cord compression and consecutive myelopathy, predominantly of the cranio-cervical junction. Disease characteristics of cranio-cervical stenosis (CCS) in MPS patients differ profoundly from other hereditary and degenerative forms. Due to high peri-procedural morbidity and mortality, MPS patients pose a substantial challenge to the unexperienced medical care provider...
April 9, 2018: Spine Journal: Official Journal of the North American Spine Society
https://www.readbyqxmd.com/read/29649527/recommendations-for-evaluation-and-management-of-pain-in-patients-with-mucopolysaccharidosis-in-latin-america
#12
J M Politei, G Gordillo-González, N Guelbert, C F M Souza, C M Lourenço, M L Solano, M M Junqueira, T S P C Magalhães, A M Martins
The mucopolysaccharidosis (MPS) constitute a heterogeneous group of rare genetic disorders caused by enzymatic deficiencies that lead to the accumulation of glycosaminoglycans (GAGs). Several types of MPS are described, historically numbered from I to IX. Clinical observations strongly suggest the presence of chronic pain in patients with all types of MPS. There are few data in the literature on the evaluation and management of pain in these patients, a fact which can compromise the quality of life even more...
April 9, 2018: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/29627477/distribution-of-mannose-receptor-in-blunt-snout-bream-megalobrama-amblycephala-during-the-embryonic-development-and-its-immune-response-to-the-challenge-of-aeromonas-hydrophila
#13
Changsong Wu, Xiaoheng Zhao, Sarath Babu V, Gailing Yuan, Weimin Wang, Jianguo Su, Xiaoling Liu, Li Lin
The mannose receptor (MR) is a type I transmembrane protein. Its ectodomain has eight C-type lectin-like domains, which are able to recognize and mediate the phagocytosis of a wide range of pathogens. Comprehensive studies have revealed that mammalian MR is widely distributed in the mononuclear phagocyte system (MPS, previously known as the reticuloendothelial system) and play a key role both in the physiological clearance and cell activation. Hitherto, neither the MR distribution, nor the function of clearance and cell activation has been investigated in fish...
April 6, 2018: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/29614328/recommendations-for-evaluation-and-management-of-pain-in-patients-with-mucopolysaccharidosis-in-latin-america
#14
J M Politei, G Gordillo-González, N Guelbert, C F M Souza, C M Lourenço, M L Solano, M M Junqueira, T S P C Magalhães, A M Martins
The mucopolysaccharidosis (MPS) constitute a heterogeneous group of rare genetic disorders caused by enzymatic deficiencies that lead to the accumulation of glycosaminoglycans (GAGs). Several types of MPS are described, historically numbered from I to IX. Clinical observations strongly suggest the presence of chronic pain in patients with all types of MPS. There are few data in the literature on the evaluation and management of pain in these patients, a fact which can compromise the quality of life even more...
March 31, 2018: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/29604577/low-levels-of-microplastics-mp-in-wild-mussels-indicate-that-mp-ingestion-by-humans-is-minimal-compared-to-exposure-via-household-fibres-fallout-during-a-meal
#15
Ana I Catarino, Valeria Macchia, William G Sanderson, Richard C Thompson, Theodore B Henry
Microplastics (MPs) are the most numerous debris reported in marine environments and assessment of the amounts of MPs that accumulate in wild organisms is necessary for risk assessment. Our objective was to assess MP contamination in mussels collected around the coast of Scotland (UK) to identify characteristics of MPs and to evaluate risk of human exposure to MPs via ingestion of mussels. We deployed caged mussels (Mytilus edulis) in an urbanised estuary (Edinburgh, UK) to assess seasonal changes in plastic pollution, and collected mussels (Mytilus spp and subtidal Modiolus modiolus) from eight sampling stations around Scotland to enumerate MP types at different locations...
March 28, 2018: Environmental Pollution
https://www.readbyqxmd.com/read/29552013/differential-phagocytic-properties-of-cd45-low-microglia-and-cd45-high-brain-mononuclear-phagocytes-activation-and-age-related-effects
#16
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
https://www.readbyqxmd.com/read/29544207/removal-of-pharmaceuticals-perfluoroalkyl-substances-and-other-micropollutants-from-wastewater-using-lignite-xylit-sand-granular-activated-carbon-gac-and-gac-polonite-%C3%A2-in-column-tests-role-of-physicochemical-properties
#17
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...
June 15, 2018: Water Research
https://www.readbyqxmd.com/read/29517765/long-term-outcomes-of-systemic-therapies-for-hurler-syndrome-an-international-multicenter-comparison
#18
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
https://www.readbyqxmd.com/read/29497418/african-trypanosomiasis-associated-anemia-the-contribution-of-the-interplay-between-parasites-and-the-mononuclear-phagocyte-system
#19
REVIEW
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
https://www.readbyqxmd.com/read/29496457/size-based-anti-tumoral-effect-of-paclitaxel-loaded-albumin-microparticle-dry-powders-for-inhalation-to-treat-metastatic-lung-cancer-in-a-mouse-model
#20
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)...
May 5, 2018: International Journal of Pharmaceutics
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