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https://www.readbyqxmd.com/read/29450922/anisotropic-thermal-and-guest-induced-responses-of-an-ultramicroporous-framework-with-rigid-linkers
#1
Josie E Auckett, Samuel G Duyker, Ekaterina I Izgorodina, Chris S Hawes, David R Turner, Stuart R Batten, Vanessa Kate Peterson
The interdependent effects of temperature and guest uptake on the structure of the ultramicroporous metal-organic framework [Cu3(cdm)4] (cdm = C(CN)2(CONH2)-) are explored in detail using in situ neutron scattering and density functional theory calculations. The tetragonal lattice displays an anisotropic thermal response related to a hinged "lattice-fence" mechanism, unusual for this topology, which is facilitated by pivoting of the rigid cdm anion about the Cu nodes. Calculated pore size metrics clearly illustrate the potential for temperature-mediated adsorption in ultramicroporous frameworks due to thermal fluctuations of the pore diameter near the value of the target guest kinetic diameter, though in [Cu3(cdm)4] this is counteracted by a competing contraction of the pore with increasing temperature as a result of the anisotropic lattice response...
February 16, 2018: Chemistry: a European Journal
https://www.readbyqxmd.com/read/29438445/crispr-cas9-in-leishmania-mexicana-a-case-study-of-lmxbtn1
#2
Aygul Ishemgulova, Jana Hlaváčová, Karolina Majerová, Anzhelika Butenko, Julius Lukeš, Jan Votýpka, Petr Volf, Vyacheslav Yurchenko
Leishmania parasites cause human cutaneous, mucocutaneous and visceral leishmaniasis. Several studies proposed involvement of certain genes in infectivity of these parasites based on differential mRNA expression data. Due to unusual gene expression mechanism, functions of such genes must be further validated experimentally. Here, we investigated a role of one of the putative virulence factors, LmxM.22.0010-encoded BTN1 (a protein involved in Batten disease in humans), in L. mexicana infectivity. Due to the incredible plasticity of the L...
2018: PloS One
https://www.readbyqxmd.com/read/29437182/videoconferencing-for-management-of-heart-failure-an-integrative-review
#3
Karen Bauce, Deborah B Fahs, Janene Batten, Robin Whittemore
Heart failure (HF) is a chronic disease associated with poor prognosis, poor quality of life (QOL), and high medical costs among older adults. Monitoring symptoms, interpreting symptoms, and decision making are self-care skills required for effective HF management. Telemonitoring (TM) is increasingly used to reduce incidence of symptom exacerbation leading to rehospitalization. An integrative review was performed to describe the efficacy of TM interventions that include videoconferencing (VC) on the HF outcomes of hospital service s use, self-care, and QOL...
February 13, 2018: Journal of Gerontological Nursing
https://www.readbyqxmd.com/read/29380022/nasal-alar-schwannoma-an-unusual-case-report
#4
Timothy C Blood, Thomas G Townes, Shaun C Desai
PURPOSE: To present the second known case of nasal alar schwannoma, first since 1973, with important considerations for surgical management. CASE REPORT: We present the case of a 25-year-old male with a 5-year history of progressively enlarging, non-tender, right alar mass with resultant ipsilateral nasal congestion and dyspnea secondary to nasal valve collapse. The mass was located cephalic to the mid-lateral aspect of the lower lateral crural cartilage, which extended to the pyriform aperture...
January 29, 2018: Aesthetic Plastic Surgery
https://www.readbyqxmd.com/read/29369397/metallothioneins-are-neuroprotective-agents-in-lysosomal-storage-disorders
#5
Eleonora Cavalca, Martina Cesani, Jennifer Gifford, Miguel Sena Esteves, Maria Rosa Terreni, Giuseppe Leoncini, Marco Peviani, Alessandra Biffi
OBJECTIVE: Lysosomal Storage Disorders (LSDs) are a broad class of inherited metabolic diseases caused by the defective activity of lysosomal enzymes. Central nervous system (CNS) manifestations are present in roughly 50% of LSD patients and represent an unmet medical need for them. We explored the therapeutic potential of Metallothioneins (MTs), a newly identified family of proteins with reported neuroprotective roles, in the murine models of two LSDs with CNS involvement. METHODS: MT-1 over-expressing transgenic mice (MTtg) were crossed with the murine models of Batten and Krabbe diseases...
January 25, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29363589/tackling-in-physical-education-rugby-an-unnecessary-risk
#6
Adam John White, John Batten, Stefan Robinson, Eric Anderson, Andrew Burns, Jo Batey, Helen Ryan-Stewart, Russell Discombe
No abstract text is available yet for this article.
January 23, 2018: Injury Prevention: Journal of the International Society for Child and Adolescent Injury Prevention
https://www.readbyqxmd.com/read/29363187/gpr65-inhibits-experimental-autoimmune-encephalomyelitis-through-cd4-t-cell-independent-mechanisms-that-include-effects-on-inkt-cells
#7
Rushika C Wirasinha, Dipti Vijayan, Nicola J Smith, Grant P Parnell, Alexander Swarbrick, Robert Brink, Cecile King, Graeme Stewart, David R Booth, Marcel Batten
The G protein-coupled receptor 65 (GPR65) gene has been genetically associated with several autoimmune diseases, including multiple sclerosis (MS). GPR65 is predominantly expressed in lymphoid organs and is activated by extracellular protons. In this study, we tested whether GPR65 plays a functional role in demyelinating autoimmune disease. Using a murine model of MS, experimental autoimmune encephalomyelitis (EAE), we found that Gpr65-deficient mice develop exacerbated disease. CD4+ helper T cells are key drivers of EAE pathogenesis, however, Gpr65 deficiency in these cells did not contribute to the observed exacerbated disease...
December 19, 2017: Immunology and Cell Biology
https://www.readbyqxmd.com/read/29345199/pulmonary-vascular-dysfunction-secondary-to-pulmonary-arterial-hypertension-insights-gained-through-retrograde-perfusion
#8
Chun Zhou, Edward S Crockett, Lynn Batten, Ivan F McMurtry, Troy Stevens
Here, we tested the hypothesis that severe pulmonary arterial hypertension impairs retrograde perfusion. To test this hypothesis, pulmonary arterial hypertension was induced in Fischer rats using a single injection of Sugen 5416 followed by 3 weeks of exposure to 10% hypoxia and then 2 weeks of normoxia. This Sugen 5416 and hypoxia regimen caused severe pulmonary arterial hypertension, with a Fulton Index of 0.73+0.07, reductions in both the pulmonary arterial acceleration time and pulmonary arterial acceleration to pulmonary arterial ejection times ratio, and extensive medial hypertrophy and occlusive neointimal lesions...
January 18, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29315659/the-role-of-glutamate-signaling-in-incentive-salience-second-by-second-glutamate-recordings-in-awake-sprague-dawley-rats
#9
Seth R Batten, Francois Pomerleau, Jorge Quintero, Greg A Gerhardt, Joshua S Beckmann
The attribution of incentive salience to reward-predictive stimuli has been shown to be associated with substance abuse-like behavior such as increased drug taking. Evidence suggests that glutamate neurotransmission and sequential N-methyl-D-aspartate (NMDA) activation are involved in the attribution of incentive salience. Here we further explore the role of second-by-second glutamate neurotransmission in the attribution of incentive salience to reward-predictive stimuli by measuring sign-tracking behavior during a Pavlovian conditioned approach procedure using ceramic-based microelectrode arrays configured for sensitive measures of extracellular glutamate in awake behaving Sprague Dawley rats...
January 8, 2018: Journal of Neurochemistry
https://www.readbyqxmd.com/read/29149460/the-impact-of-a-prolonged-stay-in-the-icu-on-patients-fundamental-care-needs
#10
Claire Minton, Lesley Batten, Annette Huntington
AIM: To explore patients', families', and health professionals' experiences of a long-stay patient in an intensive care unit. BACKGROUND: The fast-paced technologically driven intensive care unit environment, designed for a short patient stay, supports the provision of complex physiologically-focused care for those with life-threatening illnesses. Long stay patients with pronounced fundamental care needs fall outside predicted patient pathways, and nurses can find caring for these patients challenging...
November 17, 2017: Journal of Clinical Nursing
https://www.readbyqxmd.com/read/29135436/defective-synaptic-transmission-causes-disease-signs-in-a-mouse-model-of-juvenile-neuronal-ceroid-lipofuscinosis
#11
Benedikt Grünewald, Maren D Lange, Christian Werner, Aet O'Leary, Andreas Weishaupt, Sandy Popp, David A Pearce, Heinz Wiendl, Andreas Reif, Hans C Pape, Klaus V Toyka, Claudia Sommer, Christian Geis
Juvenile neuronal ceroid lipofuscinosis (JNCL or Batten disease) caused by mutations in the CLN3 gene is the most prevalent inherited neurodegenerative disease in childhood resulting in widespread central nervous system dysfunction and premature death. The consequences of CLN3 mutation on the progression of the disease, on neuronal transmission, and on central nervous network dysfunction are poorly understood. We used Cln3 knockout (Cln3(Δex1-6)) mice and found increased anxiety-related behavior and impaired aversive learning as well as markedly affected motor function including disordered coordination...
November 14, 2017: ELife
https://www.readbyqxmd.com/read/29133694/manganese-in-manganism-parkinson-s-disease-huntington-s-disease-amyotrophic-lateral-sclerosis-and-batten-disease-a-narrative-review
#12
REVIEW
Owen Proudfoot
The collective evidence to date suggests that environmental exposure to excessive amounts of manganese (Mn) can cause a neurodegenerative condition known as manganism. It is now also relatively clear that Mn is involved in the pathogenesis of Alzheimer's disease and at least some prion diseases. The potential involvement of Mn in a panel of other neurodegenerative conditions including Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, and Batten disease has been suggested and investigated, but the results to date are somewhat inconclusive...
November 2017: Neurology India
https://www.readbyqxmd.com/read/29128403/cln5-is-secreted-and-functions-as-a-glycoside-hydrolase-in-dictyostelium
#13
Robert J Huber, Sabateeshan Mathavarajah
Ceroid lipofuscinosis neuronal 5 (CLN5) is a member of a family of proteins that are linked to neuronal ceroid lipofuscinosis (NCL). This devastating neurological disorder, known commonly as Batten disease, affects all ages and ethnicities and is currently incurable. The precise function of CLN5, like many of the NCL proteins, remains to be elucidated. In this study, we report the localization, molecular function, and interactome of Cln5, the CLN5 homolog in the social amoeba Dictyostelium discoideum. Residues that are glycosylated in human CLN5 are conserved in the Dictyostelium homolog as are residues that are mutated in patients with CLN5 disease...
November 8, 2017: Cellular Signalling
https://www.readbyqxmd.com/read/29119690/experimental-infection-of-sheep-goats-and-cattle-with-a-bluetongue-virus-serotype-4-field-strain-from-bulgaria-2014
#14
C Schulz, C Sailleau, E Bréard, J Flannery, C Viarouge, S Zientara, M Beer, C Batten, B Hoffmann
In 2014, a new bluetongue virus serotype 4 (BTV-4) strain was detected in southern Greece and spread rapidly throughout the Balkan Peninsula and adjacent countries. Within half a year, more than 7,068 outbreaks were reported in ruminants, particularly in sheep. However, the reported morbidity and case fatality rates in ruminants varied. The pathogenesis of a Bulgarian BTV-4 strain isolated from sheep during the BTV-4 epizootic was studied in different species. Therefore, four sheep, three goats and three cattle were experimentally infected with the isolate BTV-4/BUL2014/15 and monitored for clinical signs up to several weeks...
November 8, 2017: Transboundary and Emerging Diseases
https://www.readbyqxmd.com/read/29117566/cytosolic-recognition-of-rna-drives-the-immune-response-to-heterologous-erythrocytes
#15
Claudia Loetsch, Joanna Warren, Adrienne Laskowski, Rodrigo Vazquez-Lombardi, Christoph Jandl, David B Langley, Daniel Christ, David R Thorburn, David K Ryugo, Jonathan Sprent, Marcel Batten, Cecile King
The archetypal T cell-dependent antigen is sheep red blood cells (SRBCs), which have defined much of what we know about humoral immunity. Early studies using solubilized or sonicated SRBCs argued that the intact structure of SRBCs was important for optimal antibody responses. However, the reason for the requirement of intact SRBCs for the response to polyvalent protein antigen remained unknown. Here, we report that the immune response to SRBCs is driven by cytosolic recognition of SRBC RNA through the RIG-I-like receptor (RLR)-mitochondrial anti-viral signaling adaptor (MAVS) pathway...
November 7, 2017: Cell Reports
https://www.readbyqxmd.com/read/29102102/bluetongue-virus-infection-in-na%C3%A3-ve-cattle-identification-of-circulating-serotypes-and-associated-culicoides-biting-midge-species-in-trinidad
#16
T Brown-Joseph, C Batten, L E Harrup, L Frost, J Flannery, H Hicks, V Ramkissoon, R Ramdeen, C V Carrington, C A L Oura
To better understand risks associated with trading cattle, it is important to know which serotypes of Bluetongue virus (BTV) are circulating within the exporting and importing country. Hence, this study was conducted to identify the circulating serotypes of BTV in Trinidad. Blood samples were collected monthly from sixty BTV- naïve imported cattle over a six month period after their arrival in the country. Virological (PCR and virus isolation) and serological (ELISA) analyses were carried out on the samples and CDC light traps were placed near the cattle enclosure to trap and identify the species of Culicoides biting midges that were present...
November 2017: Veterinary Microbiology
https://www.readbyqxmd.com/read/29097056/a-community-based-bionic-leg-rehabilitation-program-for-patients-with-chronic-stroke-clinical-trial-protocol
#17
Amy Wright, Keeron Stone, Danielle Lambrick, Simon Fryer, Lee Stoner, Edward Tasker, Simon Jobson, Grace Smith, John Batten, Jo Batey, Vicky Hudson, Helen Hobbs, James Faulkner
Stroke is a major global health problem whereby many survivors have unmet needs concerning mobility during recovery. As such, the use of robotic-assisted devices (i.e., a bionic leg) within a community setting may be an important adjunct to normal physiotherapy in chronic stroke survivors. This study will be a dual-center, randomized, parallel group clinical trial to investigate the impact of a community-based training program using a bionic leg on biomechanical, cardiovascular, and functional outcomes in stroke survivors...
October 30, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29095938/challenges-to-code-status-discussions-for-pediatric-patients
#18
Katherine E Kruse, Jason Batten, Melissa L Constantine, Saraswati Kache, David Magnus
OBJECTIVES: In the context of serious or life-limiting illness, pediatric patients and their families are faced with difficult decisions surrounding appropriate resuscitation efforts in the event of a cardiopulmonary arrest. Code status orders are one way to inform end-of-life medical decision making. The objectives of this study are to evaluate the extent to which pediatric providers have knowledge of code status options and explore the association of provider role with (1) knowledge of code status options, (2) perception of timing of code status discussions, (3) perception of family receptivity to code status discussions, and (4) comfort carrying out code status discussions...
2017: PloS One
https://www.readbyqxmd.com/read/29089465/lack-of-specificity-of-antibodies-raised-against-cln3-the-lysosomal-endosomal-transmembrane-protein-mutated-in-juvenile-batten-disease
#19
Tarah Nelson, David A Pearce, Attila D Kovacs
Juvenile CLN3 (Batten) disease, a fatal, childhood neurodegenerative disorder, results from mutations in the CLN3 gene encoding a lysosomal/endosomal transmembrane protein. The exact physiological function of CLN3 is still unknown and it is unclear how CLN3 mutations lead to selective neurodegeneration. To study the tissue expression and subcellular localization of the CLN3 protein, a number of anti-CLN3 antibodies have been generated using either the whole CLN3 protein or short peptides from CLN3 for immunization...
October 31, 2017: Bioscience Reports
https://www.readbyqxmd.com/read/29041969/glial-cells-are-functionally-impaired-in-juvenile-neuronal-ceroid-lipofuscinosis-and-detrimental-to-neurons
#20
Lotta Parviainen, Sybille Dihanich, Greg W Anderson, Andrew M Wong, Helen R Brooks, Rosella Abeti, Payam Rezaie, Giovanna Lalli, Simon Pope, Simon J Heales, Hannah M Mitchison, Brenda P Williams, Jonathan D Cooper
The neuronal ceroid lipofuscinoses (NCLs or Batten disease) are a group of inherited, fatal neurodegenerative disorders of childhood. In these disorders, glial (microglial and astrocyte) activation typically occurs early in disease progression and predicts where neuron loss subsequently occurs. We have found that in the most common juvenile form of NCL (CLN3 disease or JNCL) this glial response is less pronounced in both mouse models and human autopsy material, with the morphological transformation of both astrocytes and microglia severely attenuated or delayed...
October 17, 2017: Acta Neuropathologica Communications
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