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https://www.readbyqxmd.com/read/29326877/natural-history-of-morquio-a-patient-with-tracheal-obstruction-from-birth-to-death
#1
Caitlin Doherty, Lauren W Averill, Mary Theroux, William G Mackenzie, Christian Pizarro, Robert W Mason, Shunji Tomatsu
Morquio A syndrome (mucopolysaccharidosis IVA, MPS IVA) is a lysosomal storage disease caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase, resulting in systemic accumulation of the partially degraded glycosaminoglycans (GAGs), keratan sulfate and chondroitin-6-sulfate. The accumulation of these GAGs leads to distinguishing features as skeletal dysplasia with disproportionate dwarfism, short neck, kyphoscoliosis, pectus carinatum, tracheal obstruction, coxa valga, genu valgum, and joint laxity...
March 2018: Molecular Genetics and Metabolism Reports
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
#2
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
#3
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/29318793/adhesion-of-acanthamoeba-on-cosmetic-contact-lenses
#4
Seung Mok Lee, Ji Eun Lee, Da In Lee, Hak Sun Yu
BACKGROUND: This study aimed to evaluate the adhesion of Acanthamoeba trophozoites on cosmetic contact lenses (CLs) with and without CL care multipurpose solution (MPS) treatment. METHODS: Acanthamoeba lugdunensis L3a trophozoites were inoculated onto disks trimmed from CLs: 1-day Acuvue moist, 1-day Acuvue define, Acuvue 2, and Acuvue 2 define. After 18-hour inoculation, the number of adherent trophozoites was counted under phase contrast microscopy. The effects of MPS, Opti-Free Express, soaking CLs for 6 hours, on Acanthamoeba adhesion were analyzed...
January 22, 2018: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29279160/preoperative-assessment-of-pleural-adhesion-by-four-dimensional-ultra-low-dose-computed-tomography-4d-uldct-with-adaptive-iterative-dose-reduction-using-three-dimensional-processing-aidr-3d
#5
Masayuki Hashimoto, Yukihiro Nagatani, Yasuhiko Oshio, Norihisa Nitta, Tsuneo Yamashiro, Shinsuke Tsukagoshi, Noritoshi Ushio, Masayuki Mayumi, Tatsuya Kimoto, Tomoyuki Igarashi, Makoto Yoshigoe, Kyohei Iwai, Koki Tanaka, Shigetaka Sato, Akinaga Sonoda, Hideji Otani, Kiyoshi Murata, Jun Hanaoka
PURPOSE: To assess the feasibility of Four-Dimensional Ultra-Low-Dose Computed Tomography (4D-ULDCT) for distinguishing pleural aspects with localized pleural adhesion (LPA) from those without. METHODS: Twenty-seven patients underwent 4D-ULDCT during a single respiration with a 16cm-coverage of the body axis. The presence and severity of LPA was confirmed by their intraoperative thoracoscopic findings. A point on the pleura and a corresponding point on the outer edge of the costal bone were placed in identical axial planes at end-inspiration...
January 2018: European Journal of Radiology
https://www.readbyqxmd.com/read/29275451/voice-alterations-in-patients-with-morquio-a-syndrome
#6
Krzysztof Szklanny, Ryszard Gubrynowicz, Anna Tylki-Szymańska
Morquio A syndrome, or mucopolysaccharidosis (MPS IV A), is an inherited lysosomal storage disorder which belongs to the group of mucopolysaccharidoses (MPSs). It is caused by N-acetylgalactosamine-6-sulfatase (GALNS) activity deficiency, which results in impaired degradation of glycosaminoglycans (GAGs), including keratan sulfate (KS) and chondroitin-6-sulfate (CS). These compounds infiltrate and disrupt the architecture of the extracellular matrix, compromising the integrity of the connective tissue. Patients with Morquio A have also been noted for exhibiting abnormalities of the larynx and vocal tract...
December 23, 2017: Journal of Applied Genetics
https://www.readbyqxmd.com/read/29273298/recruitment-and-therapeutic-application-of-macrophages-in-skeletal-muscles-after-hind-limb-ischemia
#7
Pei-Ling Hsieh, Viktoriya Rybalko, Aaron B Baker, Laura J Suggs, Roger P Farrar
OBJECTIVE: Peripheral arterial disease can cause not only ischemia but also skeletal muscle damage. It has been known that macrophages (MPs) play an important role in coordinating muscle repair; however, phenotype transition of monocyte-MP in ischemic muscle has not been well defined. Hence, the purpose of this study was to examine the temporal recruitment of MPs and to explore their therapeutic effect on ischemic muscle regeneration. METHODS: Unilateral femoral artery excision was performed on C57BL/6 mice...
December 19, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/29248957/ancestry-inference-of-96-population-samples-using-microhaplotypes
#8
Ozlem Bulbul, Andrew J Pakstis, Usha Soundararajan, Cemal Gurkan, Jane E Brissenden, Janet M Roscoe, Baigalmaa Evsanaa, Ariunaa Togtokh, Peristera Paschou, Elena L Grigorenko, David Gurwitz, Sharon Wootton, Robert Lagace, Joseph Chang, William C Speed, Kenneth K Kidd
Microhaplotypes have become a new type of forensic marker with a great ability to identify and deconvolute mixtures because massively parallel sequencing (MPS) allows the alleles (haplotypes) of the multi-SNP loci to be determined directly for an individual. As originally defined, a microhaplotype locus is a short segment of DNA with two or more SNPs defining three or more haplotypes. The length is short enough, less than about 300 bp, that the read length of current MPS technology can produce a phase-known sequence of each chromosome of an individual...
December 16, 2017: International Journal of Legal Medicine
https://www.readbyqxmd.com/read/29248316/cytotoxic-and-inflammatory-effects-of-contact-lens-solutions-on-human-corneal-epithelial-cells-in-vitro
#9
Sarah Oh, David J McCanna, Lakshman N Subbaraman, Lyndon W Jones
PURPOSE: To ascertain the effect that four contact lens (CL) multipurpose solutions (MPS) have on the viability and release of pro-inflammatory cytokines from human corneal epithelial cells (HCEC). METHODS: HCEC were exposed to four different MPS at various concentrations for 18 hours. The cells were also exposed to phosphate buffer, borate buffer, and PHMB. The cell viability was evaluated using the alamarBlue assay. The release of pro-inflammatory cytokines was measured using a Multiplex electrochemiluminescent assay...
December 13, 2017: Contact Lens & Anterior Eye: the Journal of the British Contact Lens Association
https://www.readbyqxmd.com/read/29247072/vascular-and-dopaminergic-contributors-to-mild-parkinsonian-signs-in-older-adults
#10
Andrea L Rosso, Nicolaas I Bohnen, Lenore J Launer, Howard J Aizenstein, Kristine Yaffe, Caterina Rosano
OBJECTIVE: Mild parkinsonian signs (MPS) are an underappreciated neurologic condition in older adults; we assessed associations of MPS with measures of dopaminergic (catechol-O-methyltransferase [COMT] genotype, an indicator of synaptic dopamine levels) and vascular (white matter hyperintensities [WMH], an indicator of cerebral small vessel disease) factors. METHODS: In a cohort of older adults (mean age 82.6 years [SD 2.6]; 58.0% female; 38.8% black), we assessed cross-sectional associations of WMH volume and COMT Val158Met (rs4680) genotype (n = 35 Met/Met, n = 180 Val carriers) with MPS by regression models adjusted for demographic and health characteristics...
December 15, 2017: Neurology
https://www.readbyqxmd.com/read/29235046/efficiency-of-nitrogen-metabolism-in-beef-cattle-grazing-pasture-and-supplemented-with-different-protein-levels-in-the-rainy-season
#11
Renata Pereira da Silva-Marques, Joanis Tilemahos Zervoudakis, Luciana Keiko Hatamoto-Zervoudakis, Pedro Ivo José Lopes da Rosa E Silva, Núbia Bezerra do Nascimento Matos, Andresa Lazzarotto Feliciano, Rones Goulart de Paula Júnior, Luciano da Silva Cabral
Supplementation of animals in the rainy season is essential to maximise weight gain. Thus, the objective was to evaluate the effects of supplementation with low-, medium- and high-protein levels on nutritional parameters of grazing beef cattle in the rainy season. Eight rumen-cannulated Nellore bulls (437 kg body weight) were used in a 4 × 4 double Latin square design with four supplements and four experimental periods. The treatments were mineral supplement (ad libitum) and supplements with low-protein (LPSU), medium-protein (MPS) and high-protein (HPS) levels, providing 106, 408 and 601 g/day of crude protein (CP), respectively...
December 12, 2017: Tropical Animal Health and Production
https://www.readbyqxmd.com/read/29223759/methotrexate-loaded-gellan-gum-microparticles-for-drug-delivery
#12
Mukesh Dhanka, Chaitra Shetty, Rohit Srivastava
Recently, polysaccharides based microparticles have been found to offer an attractive potential as a carrier in drug delivery field. In this study, bare gellan gum microparticles (GG MPs) and methotrexate (MTX) loaded gellan gum microparticles (MTX-GG MPs) prepared by using simple water-in-oil (W/O) emulsion solvent diffusion method. The developed microparticles (MPs) were found discretely distributed in a spherical shape. MTX has been encapsulated in microparticles with 84.8±1.68% encapsulation efficiency (%EE) and 6...
December 6, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/29210605/cognitive-and-behaviour-profiles-of-children-with-mucopolysaccharidosis-type-ii
#13
Louise Crowe, Joy Yaplito-Lee, Vicki Anderson, Heidi Peters
Mucopolysaccharidosis Type II (MPS II) or Hunter Syndrome is a rare X-linked condition, due to a defect in a lysosomal enzyme involved in the breakdown of glycosaminoglycans. It is a progressive condition with worsening over time; however, symptom severity and progression rates vary. Normal intellectual function has been reported in males with mild MPS II but few studies are available that provide comprehensive cognitive profiles. Enzyme replacement therapy (ERT) can stabilize physical symptoms and has become standard treatment...
September 2017: Cognitive Neuropsychology
https://www.readbyqxmd.com/read/29173958/ion-pairing-reversed-phase-chromatography-coupled-to-inductively-coupled-plasma-mass-spectrometry-as-a-tool-to-determine-mercurial-species-in-freshwater-fish
#14
Heyong Cheng, Xiaopan Chen, Lihuan Shen, Yuanchao Wang, Zigang Xu, Jinhua Liu
Most of analytical community is focused on reversed phase high performance liquid chromatography (RP-HPLC) for mercury speciation by employing mobile phases comprising of high salts and moderate amounts of organic solvents. This study aims at rapid mercury speciation analysis by ion-pairing RP-HPLC with inductively coupled plasma mass spectrometry (ICP-MS) detection only using low salts for the sake of green analytical chemistry. Two ion-pairing HPLC methods were developed on individual usage of positively and negatively charged ion-pairing reagents (tetrabutylammonium hydroxide -TBAH and sodium dodecylbenzene sulfonate -SDBS), where sodium 3-mercapto-1-propysulfonate (MPS) and l-cysteine (Cys) were individually added in mobile phases to transform mercury species into negative and positive Hg-complexes for good resolution...
November 15, 2017: Journal of Chromatography. A
https://www.readbyqxmd.com/read/29138642/observation-of-blood-donor-recipient-malaria-parasitaemia-patterns-in-a-malaria-endemic-region
#15
Jamilu Abdullahi Faruk, Gboye Olufemi Ogunrinde, Aisha Indo Mamman
Background: Asymptomatic malaria parasitaemia has been documented in donor blood in West Africa. However, donated blood is not routinely screened for malaria parasites (MPs). The present study therefore aimed to document the frequency of blood transfusion-induced donor-recipient malaria parasitaemia patterns, in children receiving blood transfusion in a tertiary health-centre. Methodology: A cross-sectional, observational study involving 140 children receiving blood transfusion was carried out...
2017: Journal of Tropical Medicine
https://www.readbyqxmd.com/read/29137729/the-shear-wave-elastic-modulus-and-the-increased-nuclear-factor-kappa-b-nf-kb-p65-and-cyclooxygenase-2-cox-2-expression-in-the-area-of-myofascial-trigger-points-activated-in-a-rat-model-by-blunt-trauma-to-the-vastus-medialis
#16
Hengyong Lv, Zhen Li, Tiantian Hu, Yuexiang Wang, Jinpeng Wu, Yingxin Li
We aimed to elucidate the increased inflammatory cytokines expression such as nuclear factor kappa B (NF-kB/p65), cyclooxygenase-2 (COX-2), and voltage-gated calcium channels (VGCC) in the area of activated myofascial trigger points (MTrPs) in a rat model by blunt trauma to the vastus medialis and to evaluate the feasibility of a quantitative analysis of muscle elastic modulus using shear wave elastography (SWE). Twelve 7-week-old male SD rats were divided into normal (NM, n = 6) and model groups (MO, n = 6)...
November 6, 2017: Journal of Biomechanics
https://www.readbyqxmd.com/read/29126485/a-highly-sensitive-fluorescent-turn-on-biosensor-for-glycoproteins-based-on-boronic-acid-functional-polymer-capped-mn-doped-zns-quantum-dots
#17
Lifang Chang, Haocheng Wu, Xiwen He, Langxing Chen, Yukui Zhang
A simple fluorescence turn-on sensor has been designed for the highly sensitive detection of glycoproteins on the basis of boronic acid functional polymer capped Mn-doped ZnS quantum dots (QDs@MPS@AAPBA). In the absence of glycoproteins, the fluorescence emission intensity of the QDs@MPS@AAPBA was relatively weaker due to the effective electronic transfer from the QDs to the boron moieties on its surface. While the glycoproteins were introduced into the system, an obvious fluorescence enhancement was observed...
December 1, 2017: Analytica Chimica Acta
https://www.readbyqxmd.com/read/29122965/the-impact-of-dairy-protein-during-limb-immobilization-and-recovery-on-muscle-size-and-protein-synthesis-a-randomized-controlled-trial
#18
Cameron J Mitchell, Randall F D'Souza, Sarah M Mitchell, Vandre Casagrande Figueiredo, Benjamin F Miller, Karyn L Hamilton, Frederick F Peelor, Marcelli Coronet, Chantal A Pileggi, Brenan Durainayagam, Aaron C Fanning, Sally D Poppitt, David Cameron-Smith
Muscle disuse results in the loss of muscular strength and size, due to an imbalance between protein synthesis (MPS) and breakdown (MPB). Protein ingestion stimulates MPS, although it is not established if protein is able to attenuate muscle loss with immobilization (IM) or influence the recovery consisting of ambulatory movement followed by resistance training (RT). Thirty men (49.9±0.6 years) underwent 14 days of unilateral leg IM, 14 days of ambulatory recovery (AR) and a further 6 RT sessions over 14 days...
November 9, 2017: Journal of Applied Physiology
https://www.readbyqxmd.com/read/29118884/breast-arterial-calcifications-on-mammography-do-not-predict-myocardial-ischemia-on-myocardial-perfusion-single-photon-emission-computed-tomography
#19
Ahmed Fathala, Salma Salem, Fahad Alanazi, Deema Abunayyan, Abdelmoneim M Eldali, Abdulaziz Alsugair
Background: The aim of this study was to determine if breast arterial calcification (BAC) on mammography predicts myocardial ischemia (MI) on stress myocardial perfusion single-photon emission computed tomography (MPS). BAC is a type of medial artery calcification that can be seen incidentally on mammography, but the relationship between coronary artery calcification and MI on MPS is yet unknown. Methods: A total of 435 consecutive women underwent mammography and stress MPS within 1 year of each other...
October 2017: Cardiology Research
https://www.readbyqxmd.com/read/29113074/simultaneous-determination-and-investigation-of-nine-fungicides-in-fruits-using-diethylenetriamine-functional-magnetic-core-shell-polymer-modified-graphene-oxide-as-an-efficient-adsorbent-coupled-to-uplc-hrms
#20
Ming-Li Ye, Yan Zhu
In this study, diethylenetriamine-functional magnetic core-shell polymer modified graphene oxide (DETA-MPs-GO) was prepared via precipitation polymerization and amidation reaction, and it was characterized by transmission electron microscopy (TEM), Fourier-transformed infrared spectroscopy (FTIR), and X-ray diffractometer (XRD). Subsequently, a magnetic solid-phase extraction (MSPE) procedure was applied to the as-synthesized DETA-MPs-GO for the detection of nine fungicides in fruit samples, prior to ultra-performance liquid chromatography-high resolution mass spectrometry (UPLC-HRMS)...
November 5, 2017: International Journal of Molecular Sciences
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