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https://www.readbyqxmd.com/read/29160134/surfactant-protein-a-nanobody-conjugated-liposomes-loaded-with-methylprednisolone-increase-lung-targeting-specificity-and-therapeutic-effect-for-acute-lung-injury
#1
Nan Li, Dong Weng, Shan-Mei Wang, Yuan Zhang, Shan-Shan Chen, Zhao-Fang Yin, Jiali Zhai, Judy Scoble, Charlotte C Williams, Tao Chen, Hui Qiu, Qin Wu, Meng-Meng Zhao, Li-Qin Lu, Xavier Mulet, Hui-Ping Li
The advent of nanomedicine requires novel delivery vehicles to actively target their site of action. Here, we demonstrate the development of lung-targeting drug-loaded liposomes and their efficacy, specificity and safety. Our study focuses on glucocorticoids methylprednisolone (MPS), a commonly used drug to treat lung injuries. The steroidal molecule was loaded into functionalized nano-sterically stabilized unilamellar liposomes (NSSLs). Targeting functionality was performed through conjugation of surfactant protein A (SPANb) nanobodies to form MPS-NSSLs-SPANb...
November 2017: Drug Delivery
https://www.readbyqxmd.com/read/29159202/a-humoral-immune-response-alters-the-distribution-of-enzyme-replacement-therapy-in-murine-mucopolysaccharidosis-type-i
#2
Steven Q Le, Shih-Hsin Kan, Don Clarke, Valentina Sanghez, Martin Egeland, Kristen N Vondrak, Terence M Doherty, Moin U Vera, Michelina Iacovino, Jonathan D Cooper, Mark S Sands, Patricia I Dickson
Antibodies against recombinant proteins can significantly reduce their effectiveness in unanticipated ways. We evaluated the humoral response of mice with the lysosomal storage disease mucopolysaccharidosis type I treated with weekly intravenous recombinant human alpha-l-iduronidase (rhIDU). Unlike patients, the majority of whom develop antibodies to recombinant human alpha-l-iduronidase, only approximately half of the treated mice developed antibodies against recombinant human alpha-l-iduronidase and levels were low...
March 16, 2018: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/29159073/home-infusion-with-elosulfase-alpha-vimizim-r-in-a-uk-paediatric-setting
#3
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/29158997/presentation-and-treatments-for-mucopolysaccharidosis-type-ii-mps-ii-hunter-syndrome
#4
Molly Stapleton, Francyne Kubaski, Robert W Mason, Hiromasa Yabe, Yasuyuki Suzuki, Kenji E Orii, Tadao Orii, Shunji Tomatsu
Introduction: Mucopolysaccharidosis Type II (MPS II; Hunter syndrome) is an X- linked lysosomal storage disorder caused by a deficiency of iduronate-2-sulfatase (IDS). IDS deficiency leads to primary accumulation of dermatan sulfate (DS) and heparan sulfate (HS). MPS II is both multi-systemic and progressive. Phenotypes are classified as either attenuated or severe (based on absence or presence of central nervous system impairment, respectively). Areas covered: Current treatments available are intravenous enzyme replacement therapy (ERT), hematopoietic stem cell transplantation (HSCT), anti-inflammatory treatment, and palliative care with symptomatic surgeries...
2017: Expert Opinion on Orphan Drugs
https://www.readbyqxmd.com/read/29158325/mps-gather-examples-of-gps-from-outside-europe-being-unable-to-work-in-uk
#5
Abi Rimmer
No abstract text is available yet for this article.
November 20, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/29158195/understanding-missing-proteins-a-functional-perspective
#6
REVIEW
Longjian Zhou, Limsoon Wong, Wilson Wen Bin Goh
A missing protein (MP) is an unconfirmed genetic sequence for which a protein product is not yet detected. Currently, MPs are tiered based on supporting evidence mainly in the form of protein existence (PE) classification. As we discuss here, this definition is overly restrictive because proteins go missing in day-to-day proteomics as a result of low abundance, lack of sequence specificity, splice variants, and so on. Thus, we propose a broader functional classification of MPs that complements PE classification, discuss major causes, and examine three corresponding solution tiers:...
November 17, 2017: Drug Discovery Today
https://www.readbyqxmd.com/read/29155887/effects-of-an-a%C3%AE-antibody-fragment-on-a%C3%AE-aggregation-and-astrocytic-uptake-are-modulated-by-apolipoprotein-e-and-j-mimetic-peptides
#7
Laia Montoliu-Gaya, Sandra D Mulder, Robert Veerhuis, Sandra Villegas
Aβ-Immunotherapy has long been studied in the treatment of Alzheimer's disease (AD), but not how other molecules involved in the disease can affect antibody performance. We previously designed an antibody fragment, scFv-h3D6, and showed that it precludes Aβ-induced cytotoxicity by withdrawing Aβ oligomers from the amyloid pathway towards a non-toxic, worm-like pathway. ScFv-h3D6 was effective at the behavioral, cellular, and molecular levels in the 3xTg-AD mouse model. Because scFv-h3D6 treatment restored apolipoprotein E (apoE) and J (apoJ) concentrations to non-pathological values, and Aβ internalization by glial cells was found to be decreased in the presence of these apolipoproteins, we now aimed to test the influence of scFv-h3D6 on Aβ aggregation and cellular uptake by primary human astrocytes in the presence of therapeutic apoE and apoJ mimetic peptides (MPs)...
2017: PloS One
https://www.readbyqxmd.com/read/29154206/a-new-approach-in-separating-microplastics-from-environmental-samples-based-on-their-electrostatic-behavior
#8
Stefanie Felsing, Christian Kochleus, Sebastian Buchinger, Nicole Brennholt, Friederike Stock, Georg Reifferscheid
Numerous studies on microplastics (MPs; Ø < 5 mm) in the aquatic environment have been published, but knowledge about the occurrence and ecological risks of MPs is limited. This is in part because current data on the distribution of MPs are comparable only to a limited extent, due to the many different methods of investigation. In addition, sample preparation is often difficult such that standard procedures are lacking. The aim of this work was to simplify the preparation of different kinds of MP samples...
November 16, 2017: Environmental Pollution
https://www.readbyqxmd.com/read/29154057/far-infrared-ray-patches-relieve-pain-and-improve-skin-sensitivity-in-myofascial-pain-syndrome-a-double-blind-randomized-controlled-study
#9
Yen-Ting Lai, Hsiang-Lin Chan, Shu-Huan Lin, Chih-Ching Lin, Szu-Yuan Li, Chih-Kuang Liu, Hao-Wei Teng, Wen-Sheng Liu
OBJECTIVE: Myofascial pain syndrome (MPS) is a common disorder characterized by muscle pain if myofascial trigger points (MTrP) are stimulated. This study evaluated the effectiveness of far-infrared ray (FIR) patches in reducing the severity of pain in patients with MPS. METHODS: A double-blind, randomized controlled study involving 125 patients with MPS and 201 MTrPs located in the trapezius muscle. A FIR patch was applied to 98 MTrPs for 24h in the intervention group (61 patients) and a placebo patch was applied to 91 MTrPs in the control group (57 patients) at the end...
December 2017: Complementary Therapies in Medicine
https://www.readbyqxmd.com/read/29153846/surgical-management-of-neurological-manifestations-of-mucopolysaccharidosis-disorders
#10
REVIEW
Tord D Alden, Hernán Amartino, Amauri Dalla Corte, Christina Lampe, Paul R Harmatz, Leonardo Vedolin
The mucopolysaccharidosis (MPS) disorders are ultra-rare lysosomal storage disorders associated with progressive accumulation of glycosaminoglycans (GAGs) in cells and tissues throughout the body. Clinical manifestations and progression rates vary widely across and within the different types of MPS. Neurological symptoms occur frequently, and may result directly from brain damage caused by infiltration of GAGs, or develop secondary to somatic manifestations such as spinal cord compression, hydrocephalus, and peripheral nerve entrapment...
September 28, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29153844/treatment-of-brain-disease-in-the-mucopolysaccharidoses
#11
REVIEW
Maurizio Scarpa, Paul J Orchard, Angela Schulz, Patricia I Dickson, Mark E Haskins, Maria L Escolar, Roberto Giugliani
The mucopolysaccharidosis (MPS) disorders are a group of lysosomal storage diseases caused by lysosomal enzyme deficits that lead to glycosaminoglycan accumulation, affecting various tissues throughout the body based on the specific enzyme deficiency. These disorders are characterized by their progressive nature and a variety of somatic manifestations and neurological symptoms. There are established treatments for some MPS disorders, but these mostly alleviate somatic and non-neurological symptoms and do not cure the disease...
October 16, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29146990/how-gluttonous-cell-aggregates-clear-substrates-coated-with-microparticles
#12
Grégory Beaune, Andy Y W Lam, Sylvie Dufour, Françoise M Winnik, Françoise Brochard-Wyart
We study the spreading of cell aggregates deposited on adhesive substrates decorated with microparticles (MPs). A cell monolayer expands around the aggregate. The cells on the periphery of the monolayer take up the MPs, clearing the substrate as they progress and forming an aureole of cells filled with MPs. We study the dynamics of spreading and determine the width of the aureole and the level of MP internalization in cells as a function of MP size, composition, and density. From the radius and width of the aureole, we quantify the volume fraction of MPs within the cell, which leads to an easy, fast, and inexpensive measurement of the cell - particle internalization...
November 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29146369/dose-dependent-effects-of-rosmarinic-acid-on-formation-of-oxidatively-stressed-myofibrillar-protein-emulsion-gel-at-different-nacl-concentrations
#13
Shuangxi Wang, Yumeng Zhang, Lin Chen, Xinglian Xu, Guanghong Zhou, Zhixi Li, Xianchao Feng
The effects of rosmarinic acid (RA) (12, 60 and 300μM/g protein) on the textural properties and stability of oxidized myofibrillar protein (MP) emulsion gels were investigated. A low dose (12µM/g) of RA significantly prevented the loss of thiol and ε-NH2 groups and the unfolding of the oxidized MP. However, a high dose of RA (300µM/g) covalently and non-covalently interacted with the MPs, which induced a significant loss of thiol and ε-NH2 groups and aggregation of the MPs, causing decreased solubility, resulting in a poor three-dimensional emulsion gel network, and hence, higher cooking loss and lower gel strength...
March 15, 2018: Food Chemistry
https://www.readbyqxmd.com/read/29143881/rmce-based-insect-cell-platform-to-produce-membrane-proteins-captured-on-hiv-1-gag-virus-like-particles
#14
João Vidigal, Bárbara Fernandes, Mafalda M Dias, Marco Patrone, António Roldão, Manuel J T Carrondo, Paula M Alves, Ana P Teixeira
Conformationally complex membrane proteins (MPs) are therapeutic targets in many diseases, but drug discovery has been slowed down by the lack of efficient production tools. Co-expression of MPs with matrix proteins from enveloped viruses is a promising approach to obtain correctly folded proteins at the surface of virus-like particles (VLPs), preserving their native lipidic environment. Here, we implemented a site-specific recombinase-mediated cassette exchange (RMCE) strategy to establish a reusable HIV-1 Gag-expressing insect cell line for fast production of target MPs on the surface of Gag-VLPs...
November 16, 2017: Applied Microbiology and Biotechnology
https://www.readbyqxmd.com/read/29143201/newborn-screening-for-lysosomal-storage-disorders-by-tandem-mass-spectrometry-in-north-east-italy
#15
Alberto B Burlina, Giulia Polo, Leonardo Salviati, Giovanni Duro, Carmela Zizzo, Andrea Dardis, Bruno Bembi, Chiara Cazzorla, Laura Rubert, Roberta Zordan, Robert J Desnick, Alessandro P Burlina
BACKGROUND: Lysosomal storage diseases (LSDs) are inborn errors of metabolism resulting from 50 different inherited disorders. The increasing availability of treatments and the importance of early intervention have stimulated newborn screening (NBS) to diagnose LSDs and permit early intervention to prevent irreversible impairment or severe disability. We present our experience screening newborns in North East Italy to identify neonates with Mucopolysaccharidosis type I (MPS I) and Pompe, Fabry, and Gaucher diseases...
November 15, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29141507/sandwiched-white-adipose-tissue-a-novel-microphysiological-system-of-human-adipose-tissue
#16
Frank H Lau, Kelly Vogel, John P Luckett, Maxwell Hunt, Alicia Meyer, Camille L Rogers, Oren Tessler, Charles L Dupin, Hugo St Hilaire, Kazi N Islam, Trivia Frazier, Jeffrey Gimble, Steven Scahill
White adipose tissue (WAT) is a critical organ in both health and disease. However, physiologically faithful tissue culture models of primary human WAT remain limited, at best. Here we describe a novel WAT culture system in which primary human WAT is sandwiched between tissue-engineered sheets of adipose-derived stromal cells. This construct, called "sandwiched white adipose tissue" (SWAT), can be defined as a Microphysiological System (MPS) since it is a tissue-engineered, multicellular, 3D organ construct produced using human cells...
November 16, 2017: Tissue Engineering. Part C, Methods
https://www.readbyqxmd.com/read/29138642/observation-of-blood-donor-recipient-malaria-parasitaemia-patterns-in-a-malaria-endemic-region
#17
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/29137857/matricellular-proteins-and-survival-in-patients-with-pancreatic-cancer-a-systematic-review
#18
REVIEW
Sirio Fiorino, Maria Letizia Bacchi-Reggiani, Chiara Birtolo, Giorgia Acquaviva, Michela Visani, Adele Fornelli, Michele Masetti, Andrea Tura, Stefano Sbrignadello, Fabio Grizzi, Federica Patrinicola, Matteo Zanello, Laura Mastrangelo, Raffaele Lombardi, Claudia Benini, Luca Di Tommaso, Arrigo Bondi, Francesco Monetti, Elena Siopis, Paolo Emilio Orlandi, Michele Imbriani, Carlo Fabbri, Silvia Giovanelli, Andrea Domanico, Esterita Accogli, Salomone Di Saverio, Daniela Grifoni, Vincenzo Cennamo, Paolo Leandri, Elio Jovine, Dario de Biase
Extracellular matrix (ECM) plays a fundamental role in tissue architecture and homeostasis and modulates cell functions through a complex interaction between cell surface receptors, hormones, several bioeffector molecules, and structural proteins like collagen. These components are secreted into ECM and all together contribute to regulate several cellular activities including differentiation, apoptosis, proliferation, and migration. The so-called "matricellular" proteins (MPs) have recently emerged as important regulators of ECM functions...
November 4, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
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
#19
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/29137633/bi-directional-exosome-driven-intercommunication-between-the-hepatic-niche-and-cancer-cells
#20
Nikolina Dioufa, Amanda M Clark, Bo Ma, Colin H Beckwitt, Alan Wells
BACKGROUND: Our understanding of the multiple roles exosomes play during tumor progression is still very poor and the contribution of the normal tissue derived exosomes in distant seeding and tumor outgrowth has also not been widely appreciated. METHODS: Using our all-human liver microphysiological system (MPS) platform as a model to closely recapitulate the early metastatic events, we isolated exosomes from both tumor cells and liver microenvironment. RESULTS: We observed that while priming of the hepatic niche (HepN) with MDA-231 breast cancer derived exosomes facilitated seeding of the cancer cells in the liver, subsequent tumor outgrowth was diminished; this was consistent with increased entry into dormancy...
November 14, 2017: Molecular Cancer
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