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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
#1
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/29314214/oral-and-craniofacial-manifestations-in-a-hunter-syndrome-patient-with-hematopoietic-stem-cell-transplantation-a-case-report
#2
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 4, 2018: Special Care in Dentistry
https://www.readbyqxmd.com/read/29291399/the-release-of-microparticles-and-mitochondria-from-raw-264-7-murine-macrophage-cells-undergoing-necroptotic-cell-death-in-vitro
#3
Diane M Spencer, John R Dye, Claude A Pianatadosi, David S Pisetsky
Microparticles (MPs) are small membrane-bound vesicles released from activated or dying cells. As shown previously, LPS stimulation of the RAW 264.7 macrophage cell line can induce MP release, with the caspase inhibitor Z-VAD increasing the extent of this process. Since combined treatment of cells with LPS and Z-VAD can induce necroptosis, we explored particle release during this form of cell death using flow cytometry to assess particle size, binding of annexin V and staining for DNA with propidium iodide (PI) and SYTO 13...
December 29, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/29282708/p-x654r-idua-variant-among-thai-individuals-with-intermediate-mucopolysaccharidosis-type-i-and-its-residual-activity-as-demonstrated-in-cos-7-cells
#4
Lukana Ngiwsara, James R Ketudat-Cairns, Phannee Sawangareetrakul, Ratana Charoenwattanasatien, Voraratt Champattanachai, Chulaluck Kuptanon, Suthipong Pangkanon, Thipwimol Tim-Aroon, Duangrurdee Wattanasirichaigoon, Jisnuson Svasti
BACKGROUND: Mucopolysaccharidosis type I (MPS I) is a rare autosomal-recessive disorder caused by defects in alpha-L-iduronidase (IDUA), a lysosomal enzyme encoded by the IDUA gene. Herein, we characterized IDUA mutations underlying mucopolysaccharidosis type I intermediate form (Hurler-Scheie syndrome) and its molecular pathogenic mechanisms. METHODS: Clinical data, activity of the IDUA enzyme in leukocytes, and a mutation of the IDUA gene were analyzed. Pathogenesis associated with an IDUA mutation was further investigated by evaluating the mutant cDNA sequence, protein expression and activity in COS-7 cells...
December 28, 2017: Annals of Human Genetics
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
#5
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/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
#6
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/29276957/microplastics-contamination-in-molluscs-from-the-northern-part-of-the-persian-gulf
#7
Abolfazl Naji, Marzieh Nuri, A Dick Vethaak
Microplastics (MPs) are well-known emerging contaminants in the marine environment. A key route by which MPs can directly affect marine life is through ingestion. The objective of the present study was to evaluate the occurrence of MPs in marine life and seafood for human consumption in the Persian Gulf. We conducted a whole body analysis of MP (between 10 and 5000 μm in diameter) abundance in five species of molluscs with different feeding strategies, including both gastropods and bivalves from the littoral zone of the Iranian coast of the Persian Gulf...
December 22, 2017: Environmental Pollution
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
#8
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/29220051/improving-extraction-and-post-purification-concentration-of-membrane-proteins
#9
Hasin Feroz, HyeYoung Kwon, Jing Peng, Hyeonji Oh, Bryan Ferlez, Carol S Baker, John H Golbeck, Guillermo C Bazan, Andrew L Zydney, Manish Kumar
Membrane proteins (MPs), despite being critically important drug targets for the pharmaceutical industry, are difficult to study due to challenges in obtaining high yields of functional protein. Most current extraction efforts use specialized non-ionic detergents to solubilize and stabilize MPs, with MPs being concentrated by ultrafiltration (UF). However, many detergents are retained during the UF step, which can destabilize MPs and/or interfere with their characterization. Here, we studied the influence of detergent selection on the extraction and UF-based concentration of biomedically-relevant MPs, the light-driven sodium and chloride transporters, KR2 and halorhodopsin (pHR) which are also model proteins for more complex mammalian rhodopsins...
December 8, 2017: Analyst
https://www.readbyqxmd.com/read/29211120/cell-derived-microparticles-in-gingival-crevicular-fluid-from-periodontitis-patients-with-type-2-diabetes
#10
Carlos Marcelo Figueredo, Ronaldo Lira, Manuela Rubim Sete, Ricardo Guimarães Fischer
Cell-derived microparticles (MPs) have been described as vital contributors to the inflammatory process. However, its role in the periodontal disease pathogenesis remains unclear. Therefore, we aimed to detect the presence neutrophil (CD66b+) and platelet (CD41b+) derived microparticles in gingival crevicular fluid from individuals having periodontitis aggravated by type 2 diabetes. Twelve patients (56.2 ±7.2 yrs) with severe form of chronic periodontitis aggravated by type 2 diabetes were included. Clinical and metabolic data were gathered...
November 2017: Brazilian Dental Journal
https://www.readbyqxmd.com/read/29210605/cognitive-and-behaviour-profiles-of-children-with-mucopolysaccharidosis-type-ii
#11
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/29194406/whole-body-and-cns-biodistribution-of-rhhns-in-cynomolgus-monkeys-after-intrathecal-lumbar-administration-treatment-implications-for-patients-with-mps-iiia
#12
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
#13
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/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
#14
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/29132334/is-perfect-good-dimensions-of-perfectionism-in-newly-admitted-medical-students
#15
Helen Seeliger, Sigrid Harendza
BACKGROUND: Society expects physicians to perform perfectly but high levels of perfectionism are associated with symptoms of distress in medical students. This study investigated whether medical students admitted to medical school by different selection criteria differ in the occurrence of perfectionism. METHODS: Newly enrolled undergraduate medical students (n = 358) filled out the following instruments: Multidimensional Perfectionism Scale (MPS-H), Multidimensional Perfectionism Scale (MPS-F), Big Five Inventory (BFI-10), General Self-Efficacy Scale (GSE), Patient Health Questionnaire 9 (PHQ-9), and Generalized Anxiety Disorder 7 (GAD-7)...
November 13, 2017: BMC Medical Education
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
#16
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/29125954/going-deeper-into-phosphorus-adsorbents-for-lake-restoration-combined-effects-of-magnetic-particles-intraspecific-competition-and-habitat-heterogeneity-pressure-on-daphnia-magna
#17
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
#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/29097038/impact-of-the-calcium-form-of-%C3%AE-hydroxy-%C3%AE-methylbutyrate-upon-human-skeletal-muscle-protein-metabolism
#19
D J Wilkinson, T Hossain, M C Limb, B E Phillips, J Lund, J P Williams, M S Brook, J Cegielski, A Philp, S Ashcroft, J A Rathmacher, N J Szewczyk, K Smith, P J Atherton
BACKGROUND & AIMS: β-hydroxy-β-methylbutyrate (HMB) is purported as a key nutritional supplement for the preservation of muscle mass in health, disease and as an ergogenic aid in exercise. Of the two available forms of HMB (calcium (Ca-HMB) salt or free acid (FA-HMB)) - differences in plasma bioavailability have been reported. We previously reported that ∼3 g oral FA-HMB increased muscle protein synthesis (MPS) and reduced muscle protein breakdown (MPB). The objective of the present study was to quantify muscle protein metabolism responses to oral Ca-HMB...
October 6, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
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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