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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/29282638/efficacy-and-safety-of-lumen-apposing-metal-stents-in-management-of-pancreatic-fluid-collections-are-they-better-than-plastic-stents-a-systematic-review-and-meta-analysis
#3
REVIEW
Tariq Hammad, Muhammad Ali Khan, Yaseen Alastal, Wade Lee, Ali Nawras, Mohammad Kashif Ismail, Michel Kahaleh
BACKGROUND AND AIMS: Endoscopic ultrasound (EUS)-guided transmural drainage has been increasingly utilized as a first-line therapeutic modality for drainage of pancreatic fluid collections (PFC). Recently, lumen-apposing metal stents (LAMS) have been utilized for management of PFCs. We conducted a systematic review and meta-analysis to evaluate the cumulative efficacy and safety of LAMS in the management of PFC (primary outcome). We also compared the efficacy and safety of LAMS with multiple plastic stents (MPS) in the management of PFC (secondary outcome)...
December 27, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/29282529/the-development-of-skeletal-muscle-hypertrophy-through-resistance-training-the-role-of-muscle-damage-and-muscle-protein-synthesis
#4
REVIEW
Felipe Damas, Cleiton A Libardi, Carlos Ugrinowitsch
Resistance training (RT)-induced skeletal muscle hypertrophy is a highly intricate process. Despite substantial advances, we are far from understanding exactly how muscle hypertrophy develops during RT. The aim of the present review is to discuss new insights related to the role of skeletal muscle damage and muscle protein synthesis (MPS) in mediating RT-induced hypertrophy. Specifically, the thesis that in the early phase of RT (≤ 4 previous RT sessions) increases in muscle cross-sectional area are mostly attributable to muscle damage-induced muscle swelling; then (after ~ 10 sessions), a modest magnitude of muscle hypertrophy ensues; but only during a latter phase of RT (after ~ 18 sessions) is true muscle hypertrophy observed...
December 27, 2017: European Journal of Applied Physiology
https://www.readbyqxmd.com/read/29273298/recruitment-and-therapeutic-application-of-macrophages-in-skeletal-muscles-after-hind-limb-ischemia
#5
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/29248359/impact-of-long-term-elosulfase-alfa-on-activities-of-daily-living-in-patients-with-morquio-a-syndrome-in-an-open-label-multi-center-phase-3-extension-study
#6
Christian J Hendriksz, Rossella Parini, Moeenaldeen D AlSayed, Julian Raiman, Roberto Giugliani, John J Mitchell, Barbara K Burton, Norberto Guelbert, Fiona J Stewart, Derralynn A Hughes, Robert Matousek, Sara M Hawley, Celeste Decker, Paul R Harmatz
BACKGROUND: Long-term safety and efficacy of elosulfase alfa enzyme replacement therapy (ERT) were assessed in 173 patients with Morquio A syndrome (mucopolysaccharidosis IVA) in a 96-week, open-label, multi-center, phase 3 extension study (MOR-005) of the pivotal 24-week, placebo-controlled study (MOR-004). Changes in efficacy endpoints were evaluated over 120weeks, from MOR-004 baseline to MOR-005 week 96. We report the impact of ERT on activities of daily living (ADL) across three domains (mobility, self-care, and caregiver-assistance), as assessed by the Mucopolysaccharidosis Health Assessment Questionnaire (MPS-HAQ) after 72 and 120weeks or approximately 1 and 2years...
December 5, 2017: Molecular Genetics and Metabolism
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
#7
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/29219321/sheta2-dry-powder-aerosols-for-tuberculosis-formulation-design-and-optimization-using-quality-by-design
#8
Mariam Ibrahim, Manolya Kukut Hatipoglu, Lucila Garcia-Contreras
Tuberculosis (TB) is a life threatening pulmonary infection caused by Mycobacterium tuberculosis (MTB). Current treatments are complex, lengthy and associated with severe side effects that decrease patient compliance and increase the probability of the emergence of drug resistant strains. Thus, more effective drugs with little to no side effects are needed to diversify the armamentarium against the global TB epidemic. SHetA2, an anticancer compound with null toxicity at doses much higher than the effective dose, was recently discovered to be active against MTB...
December 8, 2017: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/29194406/whole-body-and-cns-biodistribution-of-rhhns-in-cynomolgus-monkeys-after-intrathecal-lumbar-administration-treatment-implications-for-patients-with-mps-iiia
#9
Jou-Ku Chung, Luying Pan, Kathleen Palmieri, Amir S Youssef, Thomas G McCauley
Mucopolysaccharidosis III type A (MPS IIIA; Sanfilippo syndrome), a genetic lysosomal disorder causing a deficiency of heparan N-sulfatase (HNS), leads to progressive cognitive decline from an early age. An effective enzyme replacement therapy (ERT) for MPS IIIA requires central nervous system (CNS) biodistribution. Recombinant human heparan N-sulfatase (rhHNS), an investigatory ERT for MPS IIIA, has been formulated for intrathecal (IT) administration since intravenous (IV) administration cannot cross the blood brain barrier (BBB) in sufficient amounts to have a therapeutic effect...
December 1, 2017: International Journal of Molecular Sciences
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
#10
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/29170142/a-robust-microparticle-platform-for-a-sting-targeted-adjuvant-that-enhances-both-humoral-and-cellular-immunity-during-vaccination
#11
Robert D Junkins, Matthew D Gallovic, Brandon M Johnson, Michael A Collier, Rebekah Watkins-Schulz, Ning Cheng, Clément N David, Charles E McGee, Gregory D Sempowski, Ivo Shterev, Karen McKinnon, Eric M Bachelder, Kristy M Ainslie, Jenny P-Y Ting
Most FDA-approved adjuvants for infectious agents boost humoral but not cellular immunity, and have poorly-understood mechanisms. Stimulator of interferon genes (STING, also known as MITA, MPYS, or ERIS) is an exciting adjuvant target due to its role in cyclic dinucleotide (CDN)-driven anti-viral immunity; however, a major hindrance is STING's cytosolic localization which requires intracellular delivery of its agonists. As a result, STING agonists administered in a soluble form have elicited suboptimal immune responses...
November 20, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/29159073/home-infusion-with-elosulfase-alpha-vimizim-r-in-a-uk-paediatric-setting
#12
Niamh Finnigan, Jane Roberts, Jean Mercer, Simon A Jones
Enzyme replacement therapy is the only available treatment for Mucopolysaccharidosis type IVA (MPS IVA, Morquio syndrome). The treatment is lengthy and invasive involving weekly intravenous infusions of 4-5 h. This can cause significant disruption to normal family life so the provision of a safe and effective homecare service is essential. In order to deliver a safe service, robust standards must be in place; this includes appropriately trained members of homecare staff, detailed management for infusion related reactions (IRR) and appropriate venous access...
March 2018: Molecular Genetics and Metabolism Reports
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
#13
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/29125954/going-deeper-into-phosphorus-adsorbents-for-lake-restoration-combined-effects-of-magnetic-particles-intraspecific-competition-and-habitat-heterogeneity-pressure-on-daphnia-magna
#14
Ana Del Arco, Gema Parra, Inmaculada de Vicente
Aquatic population responses to chemical exposure may be exacerbated by intraspecific competition pressures, being also shaped by habitat heterogeneity. Magnetic particles (MPs) have been recently proposed as promising phosphorus (P) adsorbents for lake restoration. This study focuses on assessing the effects of MPs on the abundance of the crustacean Daphnia magna under different levels of both intraspecific competition pressure and habitat heterogeneity. The experimental design consisted of two experiments (in homogeneous and heterogeneous habitats) done in glass jars with four concentrations of MPs: controls of 0g MPsL(-1), and treatments of 1, 1...
November 7, 2017: Ecotoxicology and Environmental Safety
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
#15
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/29122734/gene-editing-of-mps-i-human-fibroblasts-by-co-delivery-of-a-crispr-cas9-plasmid-and-a-donor-oligonucleotide-using-nanoemulsions-as-nonviral-carriers
#16
Roselena Silvestri Schuh, Talita Giacomet de Carvalho, Roberto Giugliani, Ursula Matte, Guilherme Baldo, Helder Ferreira Teixeira
Mucopolysaccharidosis type I (MPS I) is an inherited disease caused by the deficiency of alpha-L-iduronidase (IDUA). This study shows the use of nanoemulsions co-complexed with the plasmid of CRISPR/Cas9 system and a donor oligonucleotide aiming at MPS I gene editing in vitro. Nanoemulsions composed of MCT, DOPE, DOTAP, DSPE-PEG, and water were prepared by high-pressure homogenization. The DNA was complexed by adsorption or encapsulation of preformed DNA/DOTAP complexes with nanoemulsions at +4/ -1 charge ratio...
November 6, 2017: European Journal of Pharmaceutics and Biopharmaceutics
https://www.readbyqxmd.com/read/29110472/enzymatic-purification-of-microplastics-in-environmental-samples
#17
Martin G J Löder, Hannes Klaus Imhof, Maike Ladehoff, Lena A Löschel, Claudia Lorenz, Svenja Mintenig, Sarah Piehl, Sebastian Primpke, Isabella Schrank, Christian Laforsch, Gunnar Gerdts
Micro-Fourier transform infrared (micro-FTIR) spectroscopy and Raman spectroscopy enable the reliable identification and quantification of microplastics (MPs) in the lower micron range. Since concentrations of MPs in the environment are usually low, the large sample volumes required for these techniques lead to an excess of co-enriched organic or inorganic materials. While inorganic materials can be separated from MPs using density separation, the organic fraction impedes the ability to conduct reliable analyses...
November 7, 2017: Environmental Science & Technology
https://www.readbyqxmd.com/read/29102761/a-rapid-scoring-tool-to-assess-mutation-probability-in-patients-with-inherited-cardiac-disorders
#18
Dominica Zentner, Tina Thompson, Jessica Taylor, Michael Bogwitz, Alison Trainer, Jitendra Vohra, Ingrid Winship, Paul A James
AIMS: To explore clinical features and relationship with positive mutation ascertainment in inherited heart diseases in order to develop a clinical tool to assist identification of individuals in whom to offer genetic testing. A clinical tool that increases pre test probability of mutation detection would have the benefits of improving patient counselling, prioritising cases for MPS and allowing equity in decision making. METHODS AND RESULTS: Consecutive MPS mutation detection testing cases were identified (September 2014 - December 2015, n = 126)...
November 1, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29099523/a-potential-strategy-for-counteracting-age-related-sarcopenia-preliminary-evidence-of-combined-exercise-training-and-leucine-supplementation
#19
Z Xia, J M Cholewa, Y Zhao, Y Yang, H Shang, H Jiang, Q Su, N E Zanchi
Previous research has demonstrated the positive effects of concurrent/combined aerobic and resistance exercise or leucine supplementation on skeletal muscle protein synthesis (MPS) and hypertrophy in aging organisms. However, the effects of a multimodal intervention which combines both aerobic and resistance exercise and leucine supplementation has not been fully elucidated. Eighteen month old and 2 month old C57BL/6 mice were assigned to aging control (AC, n = 8), aging and multimodal intervention (AMI, n = 8) and young control (YC, n = 8)...
December 13, 2017: Food & Function
https://www.readbyqxmd.com/read/29095970/failed-recovery-of-glycemic-control-and-myofibrillar-protein-synthesis-with-two-weeks-of-physical-inactivity-in-overweight-pre-diabetic-older-adults
#20
Chris McGlory, Mark T von Allmen, Tanner Stokes, Robert Given Morton, Amy J Hector, Briony A Lago, Amogelang R Raphenya, Brennan K Smith, Andrew G McArthur, Gregory R Steinberg, Steven K Baker, Stuart M Phillips
Background: Physical inactivity impairs insulin sensitivity, which is exacerbated with aging. We examined the impact of two weeks of acute inactivity and recovery on glycemic control, integrated rates of muscle protein synthesis (MPS) in older men and women. Methods: Twenty-two overweight, pre-diabetic older adults (12 men, 10 women, 69 ± 4 yr) undertook 7 d of habitual activity (baseline; BL), step reduction (SR; <1000 steps .d-1 for 14 d), followed by 14d of recovery (RC)...
October 31, 2017: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
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