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https://www.readbyqxmd.com/read/29346563/lc3-immunostaining-in-the-inferior-olivary-nuclei-of-cats-with-niemann-pick-disease-type-c1-is-associated-with-patterned-purkinje-cell-loss
#1
Brittney L Gurda, Jessica H Bagel, Samantha J Fisher, Mark L Schultz, Andrew P Lieberman, Peter Hand, Charles H Vite, Gary P Swain
The feline model of Niemann-Pick disease, type C1 (NPC1) recapitulates the clinical, neuropathological, and biochemical abnormalities present in children with NPC1. The hallmarks of disease are the lysosomal storage of unesterified cholesterol and multiple sphingolipids in neurons, and the spatial and temporal distribution of Purkinje cell death. In feline NPC1 brain, microtubule-associated protein 1 light chain 3 (LC3) accumulations, indicating autophagosomes, were found within axons and presynaptic terminals...
January 13, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29340494/niemann-pick-disease-type-b-hrct-assessment-of-pulmonary-involvement
#2
Heloisa Maria Pereira Freitas, Alexandre Dias Mançano, Rosana Souza Rodrigues, Bruno Hochhegger, Pedro Paulo Teixeira E Silva Torres, Dante Escuissato, Cesar Augusto Araujo Neto, Edson Marchiori
OBJECTIVE: To analyze HRCT findings in patients with Niemann-Pick disease (NPD) type B, in order to determine the frequency of HRCT patterns and their distribution in the lung parenchyma, as well as the most common clinical characteristics. METHODS: We studied 13 patients (3 males and 10 females) aged 5 to 56 years. HRCT images were independently evaluated by two observers, and disagreements were resolved by consensus. The inclusion criteria were presence of abnormal HRCT findings and diagnosis of NPD type B confirmed by histopathological examination of a bone marrow, lung, or liver biopsy specimen...
November 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/29326013/a-cluster-of-multidrug-resistant-mycobacterium-tuberculosis-among-patients-arriving-in-europe-from-the-horn-of-africa-a-molecular-epidemiological-study
#3
Timothy M Walker, Matthias Merker, Astrid M Knoblauch, Peter Helbling, Otto D Schoch, Marieke J van der Werf, Katharina Kranzer, Lena Fiebig, Stefan Kröger, Walter Haas, Harald Hoffmann, Alexander Indra, Adrian Egli, Daniela M Cirillo, Jérôme Robert, Thomas R Rogers, Ramona Groenheit, Anne T Mengshoel, Vanessa Mathys, Marjo Haanperä, Dick van Soolingen, Stefan Niemann, Erik C Böttger, Peter M Keller
BACKGROUND: The risk of tuberculosis outbreaks among people fleeing hardship for refuge in Europe is heightened. We describe the cross-border European response to an outbreak of multidrug-resistant tuberculosis among patients from the Horn of Africa and Sudan. METHODS: On April 29 and May 30, 2016, the Swiss and German National Mycobacterial Reference Laboratories independently triggered an outbreak investigation after four patients were diagnosed with multidrug-resistant tuberculosis...
January 8, 2018: Lancet Infectious Diseases
https://www.readbyqxmd.com/read/29325023/the-extending-spectrum-of-npc1-related-human-disorders-from-niemann-pick-c1-disease-to-obesity
#4
Amel Lamri, Marie Pigeyre, William S Garver, David Meyre
The Niemann-Pick C1 (NPC1) protein regulates the transport of cholesterol and fatty acids from late endosomes / lysosomes and has a central role in maintaining lipid homeostasis. NPC1 loss-of-function mutations in humans cause NPC1 disease, a rare autosomal-recessive lipid-storage disorder characterized by progressive and lethal neurodegeneration, liver and lung failure, due to cholesterol infiltration. In humans, genome wide association studies (GWAS) and post-GWAS reports highlight the implication of common variants in NPC1 in adult-onset obesity, body fat mass, and type 2 diabetes...
January 9, 2018: Endocrine Reviews
https://www.readbyqxmd.com/read/29324225/the-marburgvirus-neutralizing-human-monoclonal-antibody-mr191-targets-a-conserved-site-to-block-virus-receptor-binding
#5
Liam B King, Marnie L Fusco, Andrew I Flyak, Philipp A Ilinykh, Kai Huang, Bronwyn Gunn, Robert N Kirchdoerfer, Kathryn M Hastie, Amandeep K Sangha, Jens Meiler, Galit Alter, Alexander Bukreyev, James E Crowe, Erica Ollmann Saphire
Since their first identification 50 years ago, marburgviruses have emerged several times, with 83%-90% lethality in the largest outbreaks. Although no vaccines or therapeutics are available for human use, the human antibody MR191 provides complete protection in non-human primates when delivered several days after inoculation of a lethal marburgvirus dose. The detailed neutralization mechanism of MR191 remains outstanding. Here we present a 3.2 Å crystal structure of MR191 complexed with a trimeric marburgvirus surface glycoprotein (GP)...
January 10, 2018: Cell Host & Microbe
https://www.readbyqxmd.com/read/29321555/fourier-transform-infrared-microscopy-enables-guidance-of-automated-mass-spectrometry-imaging-to-predefined-tissue-morphologies
#6
Jan-Hinrich Rabe, Denis A Sammour, Sandra Schulz, Bogdan Munteanu, Martina Ott, Katharina Ochs, Peter Hohenberger, Alexander Marx, Michael Platten, Christiane A Opitz, Daniel S Ory, Carsten Hopf
Multimodal imaging combines complementary platforms for spatially resolved tissue analysis that are poised for application in life science and personalized medicine. Unlike established clinical in vivo multimodality imaging, automated workflows for in-depth multimodal molecular ex vivo tissue analysis that combine the speed and ease of spectroscopic imaging with molecular details provided by mass spectrometry imaging (MSI) are lagging behind. Here, we present an integrated approach that utilizes non-destructive Fourier transform infrared (FTIR) microscopy and matrix assisted laser desorption/ionization (MALDI) MSI for analysing single-slide tissue specimen...
January 10, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29315901/synthesis-and-reactivity-of-the-donor-stabilized-bis-pentafluoro-ethyl-stannylene-sn-c2f5-2-d-n-d-thf-dmap-pme3-sn-c2f5-3
#7
Johannes Klösener, Markus Wiesemann, Mark Niemann, Beate Neumann, Hans-Georg Stammler, Berthold Hoge
In this contribution we report on the synthesis of bis(pentafluoroethyl)stannane, H2Sn(C2F5)2, and its conversion to the corresponding bis(pentafluoroethyl)stannylene, Sn(C2F5)2. This highly reactive novel stannylene exists in the monomeric form when stabilized by a suitable donor, [Sn(C2F5)2(D)n] (D = THF, DMAP, PMe3, [Sn(C2F5)3]-). In the absence of donor molecules the formation of oligomeric and cyclic stannylene moieties was observed [Sn(C2F5)2]n. The reaction with bimetallic compounds leads to a donor-depending stannylene or distannylene insertion into the metal-metal bond...
January 9, 2018: Chemistry: a European Journal
https://www.readbyqxmd.com/read/29313356/sub-cellular-nanorheology-reveals-lysosomal-viscosity-as-a-reporter-for-lysosomal-storage-diseases
#8
John Devany, Kasturi Chakraborty, Yamuna Krishnan
We describe a new method to measure viscosity within sub-cellular organelles of a living cell using nanorheology. We demonstrate proof of concept by measuring viscosity in lysosomes in multiple cell types and disease models. The lysosome is an organelle, which is responsible for the breakdown of complex biomolecules. When different lysosomal proteins are defective, they are unable to breakdown specific biological substrates, that get stored within the lysosome, causing about 70 fatal diseases called lysosomal storage disorders (LSDs)...
January 9, 2018: Nano Letters
https://www.readbyqxmd.com/read/29311470/associations-between-lifestyle-related-diseases-and-transporters-involved-in-intestinal-absorption-and-biliary-excretion-of-cholesterol
#9
Yoshihide Yamanashi, Tappei Takada, Hiroshi Suzuki
Westernization of dietary habits leads to an increase in lipid intake and is thought to be responsible for an increase in patients with dyslipidemia. It is a well-known fact that the impaired cholesterol homeostasis is closely related to the development of various lifestyle-related diseases such as fatty liver, diabetes, and gallstone as well as dyslipidemia leading to atherosclerosis and cardiovascular diseases such as heart attack and stroke. Therefore, appropriate management of cholesterol levels in the body is considered important in prevention and treatments of these lifestyle-related diseases and in addition, molecular mechanisms controlling plasma (and/or hepatic) cholesterol levels have been intensively studied...
2018: Biological & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/29310502/post-mortem-computed-tomography-technical-principles-and-recommended-parameter-settings-for-high-resolution-imaging
#10
Dominic Gascho, Michael J Thali, Tilo Niemann
Post-mortem computed tomography (PMCT) has become a standard procedure in many forensic institutes worldwide. However, the standard scan protocols offered by vendors are optimised for clinical radiology and its main considerations regarding computed tomography (CT), namely, radiation exposure and motion artefacts. Thus, these protocols aim at low-dose imaging and fast imaging techniques. However, these considerations are negligible in post-mortem imaging, which allows for significantly increased image quality...
January 1, 2018: Medicine, Science, and the Law
https://www.readbyqxmd.com/read/29304874/total-arterial-revascularization-in-patients-with-acute-myocardial-infarction-feasibility-and-outcomes
#11
Philippe Grieshaber, Lukas Oster, Tobias Schneider, Victoria Johnson, Coskun Orhan, Peter Roth, Bernd Niemann, Andreas Böning
BACKGROUND: In acute situations such as acute myocardial infarction (AMI) with indication for coronary artery bypass grafting (CABG), total arterial revascularization (TAR) is often rejected in favour of saphenous vein (SV) grafting, which is assumed to allow for quicker vessel harvesting, a simpler anastomosis technique, and thus quicker revascularization and fewer bleeding complications. The aim of this study was to evaluate whether reluctance to apply TAR in AMI is still justified from a technical point of view in the current era and whether superiority of TAR results is also evident in emergency patients with AMI undergoing CABG...
January 5, 2018: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29304405/targeting-polysialic-acid-abundant-cancers-using-oncolytic-adenoviruses-with-fibers-fused-to-active-bacteriophage-borne-endosialidase
#12
Nikolas T Martin, Christoph Wrede, Julia Niemann, Jennifer Brooks, David Schwarzer, Florian Kühnel, Rita Gerardy-Schahn
Genetic replacement of adenoviral fiber knobs by ligands that enable tumor specific targeting of oncolytic adenoviruses is challenging because the fiber knob contributes to virus assembly. Here, we present a novel concept by describing stable recombinant adenoviruses with tumor specific infection mode. The fiber knob was replaced by endosialidaseNF (endoNF), the tailspike protein of bacteriophage K1F. EndoNF recognizes polysialic acid, an oncofetal antigen characteristic for high malignant tumors of neuroendocrine origin...
December 12, 2017: Biomaterials
https://www.readbyqxmd.com/read/29301955/the-cytokinin-oxidase-dehydrogenase-ckx1-is-a-membrane-bound-protein-requiring-homooligomerization-in-the-endoplasmic-reticulum-for-its-cellular-activity
#13
Michael C E Niemann, Henriette Weber, Tomáš Hluska, Georgeta Leonte, Samantha M Anderson, Ondrej Novak, Alessandro Senes, Tomas Werner
Degradation of the plant hormone cytokinin is controlled by cytokinin oxidase/dehydrogenase (CKX) enzymes. The molecular and cellular behavior of these proteins is still largely unknown. In this study, we show that CKX1 is a type-II single-pass membrane protein that predominantly localizes to the endoplasmic reticulum (ER). This indicates that this CKX isoform is a bona fide ER protein directly controlling the cytokinin which triggers the signaling from the ER. By using various approaches, we demonstrate that CKX1 forms homodimers and homooligomers in vivo...
January 4, 2018: Plant Physiology
https://www.readbyqxmd.com/read/29300152/different-effects-of-two-mutations-on-the-infectivity-of-ebola-virus-glycoprotein-in-nine-mammalian-species
#14
Yohei Kurosaki, Mahoko Takahashi Ueda, Yusuke Nakano, Jiro Yasuda, Yoshio Koyanagi, Kei Sato, So Nakagawa
Ebola virus (EBOV), which belongs to the genus Ebolavirus, causes a severe and often fatal infection in primates, including humans, whereas Reston virus (RESTV) only causes lethal disease in non-human primates. Two amino acids (aa) at positions 82 and 544 of the EBOV glycoprotein (GP) are involved in determining viral infectivity. However, it remains unclear how these two aa residues affect the infectivity of Ebolavirus species in various hosts. Here we performed viral pseudotyping experiments with EBOV and RESTV GP derivatives in 10 cell lines from 9 mammalian species...
January 4, 2018: Journal of General Virology
https://www.readbyqxmd.com/read/29299098/that-s-my-styleoideum-symptomatic-os-styloideum-in-an-adolescent-male
#15
Malwina Kaniewska, Mathias Haefeli, Urs Laesser, Tilo Niemann
We present a 15-year-old male patient with persistent localized pain on the dorsal side of the left wrist between the base of the 2nd and 3rd metacarpal bones and over the third carpometacarpal joint. It was diagnosed as an accessory metacarpal bone -Os styloideum. This entity may be detected on plain radiographs and in ultrasound examination and is often asymptomatic. Symptomatic os styloideum occurs more frequently in the dominant hand and may be treated conservatively with corticosteroid infiltration. A palpable prominence on the dorsal side of the wrist and focal pain evoked anxiety of the adolescent patient who searched medical consultation...
July 2017: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/29290526/rapid-screening-for-lipid-storage-disorders-using-biochemical-markers-expert-center-data-and-review-of-the-literature
#16
M Voorink-Moret, S M I Goorden, A B P van Kuilenburg, F A Wijburg, J M M Ghauharali-van der Vlugt, F S Beers-Stet, A Zoetekouw, W Kulik, C E M Hollak, F M Vaz
BACKGROUND: In patients suspected of a lipid storage disorder (sphingolipidoses, lipidoses), confirmation of the diagnosis relies predominantly on the measurement of specific enzymatic activities and genetic studies. New UPLC-MS/MS methods have been developed to measure lysosphingolipids and oxysterols, which, combined with chitotriosidase activity may represent a rapid first tier screening for lipid storage disorders. MATERIAL AND METHODS: A lysosphingolipid panel consisting of lysoglobotriaosylceramide (LysoGb3), lysohexosylceramide (LysoHexCer: both lysoglucosylceramide and lysogalactosylceramide), lysosphingomyelin (LysoSM) and its carboxylated analogue lysosphingomyelin-509 (LysoSM-509) was measured in control subjects and plasma samples of predominantly untreated patients affected with lipid storage disorders (n=74)...
December 22, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29285780/axially-decorated-siiv-phthalocyanines-bearing-mannose-or-ammonium-conjugated-siloxanes-comparative-bacterial-labelling-and-photodynamic-inactivation
#17
Malte C Grüner, Silke Niemann, Andreas Faust, Cristian A Strassert
Herein we present a comparative study about the photoinactivation of Staphylococcus aureus (Gram-positive model) and Escherichia coli (Gram-negative model) employing a neutral and a dicationic axially functionalized SiIV phthalocyanine. Depending on the charge of the siloxane moiety (neutral monosaccharide or cationic ammonium salt), different interactions with the bacteria were observed and a differential photoinactivation was facilitated. The intensity of the fluorescence labelling correlated with the photoinactivation of the two types of bacteria: while the neutral species only significantly affected the Gram-positive cells, we observed that the positively charged photosensitizer interacted both with the Gram-positive and with the Gram-negative models...
December 29, 2017: Photochemistry and Photobiology
https://www.readbyqxmd.com/read/29284687/a-standardised-method-for-interpreting-the-association-between-mutations-and-phenotypic-drug-resistance-in-mycobacterium-tuberculosis
#18
Paolo Miotto, Belay Tessema, Elisa Tagliani, Leonid Chindelevitch, Angela M Starks, Claudia Emerson, Debra Hanna, Peter S Kim, Richard Liwski, Matteo Zignol, Christopher Gilpin, Stefan Niemann, Claudia M Denkinger, Joy Fleming, Robin M Warren, Derrick Crook, James Posey, Sebastien Gagneux, Sven Hoffner, Camilla Rodrigues, Iñaki Comas, David M Engelthaler, Megan Murray, David Alland, Leen Rigouts, Christoph Lange, Keertan Dheda, Rumina Hasan, Uma Devi K Ranganathan, Ruth McNerney, Matthew Ezewudo, Daniela M Cirillo, Marco Schito, Claudio U Köser, Timothy C Rodwell
A clear understanding of the genetic basis of antibiotic resistance in Mycobacterium tuberculosis is required to accelerate the development of rapid drug susceptibility testing methods based on genetic sequence.Raw genotype-phenotype correlation data were extracted as part of a comprehensive systematic review to develop a standardised analytical approach for interpreting resistance associated mutations for rifampicin, isoniazid, ofloxacin/levofloxacin, moxifloxacin, amikacin, kanamycin, capreomycin, streptomycin, ethionamide/prothionamide and pyrazinamide...
December 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29276852/organ-donor-management-part-1-toward-a-consensus-to-guide-anesthesia-services-during-donation-after-brain-death
#19
Michael J Souter, E Eidbo, James Y Findlay, Daniel J Lebovitz, Marina Moguilevitch, Nikole A Neidlinger, Gerhard Wagener, Anil S Paramesh, Claus U Niemann, Pamela R Roberts, Ernesto A Pretto
Worldwide 715 482 patients have received a lifesaving organ transplant since 1988. During this time, there have been advances in donor management and in the perioperative care of the organ transplant recipient, resulting in marked improvements in long-term survival. Although the number of organs recovered has increased year after year, a greater demand has produced a critical organ shortage. The majority of organs are from deceased donors; however, some are not suitable for transplantation. Some of this loss is due to management of the donor...
December 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29275369/regional-call-911-emergency-department-protocol-to-reduce-interfacility-transfer-delay-for-patients-with-st-segment-elevation-myocardial-infarction
#20
Nichole Bosson, Terrence Baruch, William J French, Andrea Fang, Amy H Kaji, Marianne Gausche-Hill, Alisa Rock, David Shavelle, Joseph L Thomas, James T Niemann
BACKGROUND: We evaluated the first-medical-contact-to-balloon (FMC2B) time after implementation of a "Call 911" protocol for ST-segment-elevation myocardial infarction (STEMI) interfacility transfers in a regional system. METHODS AND RESULTS: This is a retrospective cohort study of consecutive patients with STEMI requiring interfacility transfer from a STEMI referring hospital, to one of 35 percutaneous coronary intervention-capable STEMI receiving centers (SRCs)...
December 23, 2017: Journal of the American Heart Association
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