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https://www.readbyqxmd.com/read/29142099/assessing-tms-induced-d-and-i-waves-with-spinal-h-reflexes
#1
Niclas Niemann, Patrick Wiegel, Alexander Kurz, John C Rothwell, Christian Leukel
Transcranial magnetic stimulation (TMS) of motor cortex produces a series of descending volleys known as D- (direct) and I- (indirect) waves. In the present study, we questioned whether spinal H-reflexes can be used to dissect D-waves, early and late I-waves from TMS. We therefore probed H-reflex facilitation at arrival times of D- and I-waves at the spinal level and thereby changed TMS parameters that have previously been shown to have selective effects on evoked D- and different I-waves. We changed TMS intensity and current direction, and applied a double-pulse paradigm known as short-interval intracortical inhibition (SICI)...
November 15, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/29139343/mycobacterium-abscessus-a-taxonomic-puzzle
#2
Enrico Tortoli, Tomas A Kohl, Barbara A Brown-Elliott, Alberto Trovato, Sylvia Cardoso-Leão, Maria Jesus Garcia, Sruthi Vasireddy, Christine Y Turenne, David E Griffith, Julie V Philley, Stefan Niemann, Richard J Wallace, Daniela M Cirillo
No abstract text is available yet for this article.
November 15, 2017: International Journal of Systematic and Evolutionary Microbiology
https://www.readbyqxmd.com/read/29138063/polyrotaxane-based-systemic-delivery-of-%C3%AE-cyclodextrins-for-potentiating-therapeutic-efficacy-in-a-mouse-model-of-niemann-pick-type-c-disease
#3
Atsushi Tamura, Nobuhiko Yui
Niemann-Pick type C (NPC) disease is a fatal metabolic disorder characterized by the lysosomal accumulation of cholesterol. Although 2-hydroxypropyl β-cyclodextrin (HP-β-CD) promotes the excretion of cholesterol and prolongs the life span in animal models of NPC disease, it requires extremely high dose. We developed acid-labile β-CD-based polyrotaxanes (PRXs) comprising multiple β-CDs threaded along a polymer chain capped with acid-cleavable stopper molecules for potentiating therapeutic efficacy of β-CD in NPC disease...
November 11, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/29135293/-from-the-hallervorden-spatz-eponym-to-the-molecular-terminology
#4
Ferenc Garzuly
INTRODUCTION AND AIM: A combination of Niemann-Pick- and Hallervorden-Spatz diseases led to the death of a 17-year-old boy in 1994. Genetic counseling necessitated further investigations in 2017. Meanwhile, the nomenclature of Hallervorden-Spatz disease has been abandoned. The author analyze the reasons for this change. METHOD: Professional activities of Hallervorden and Spatz during and after the Nazi euthanasia program are presented. Also, the scientific efforts that led to the discovery of the genetic background of the disease and ultimately to its new name are highlighted...
October 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/29133554/what-is-resistance-impact-of-phenotypic-versus-molecular-drug-resistance-testing-on-multi-and-extensively-drug-resistant-tuberculosis-therapy
#5
Jan Heyckendorf, Sönke Andres, Claudio U Köser, Ioana D Olaru, Thomas Schön, Erik Sturegård, Patrick Beckert, Viola Schleusener, Thomas A Kohl, Doris Hillemann, Danesh Moradigaravand, Julian Parkhill, Sharon J Peacock, Stefan Niemann, Christoph Lange, Matthias Merker
Rapid and accurate drug-susceptibility testing (DST) is essential for the treatment of multi- and extensively drug-resistant tuberculosis (M/XDR-TB). We compared the utility of genotypic DST assays with phenotypic DST (pDST) using BACTEC 960 MGIT or Löwenstein-Jensen to construct M/XDR-TB treatment regimens for a cohort of 25 consecutive M/XDR-TB patients and 15 possible anti-TB drugs.Genotypic DST results from Cepheid GeneXpert MTB/RIF (Xpert) and line probe assays (LPAs: Hain GenoType MTBDRplus 2.0 and MTBDRsl 2...
November 13, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/29133246/potentially-inappropriate-liver-transplantation-in-the-era-of-the-sickest-first-policy-a-search-for-the-upper-limits
#6
REVIEW
Michael Linecker, Tanja Krones, Thomas Berg, Claus U Niemann, Randolph H Steadman, Philipp Dutkowski, Pierre-Alain Clavien, Ronald W Busuttil, Robert D Truog, Henrik Petrowsky
Liver transplantation has emerged to a highly efficient treatment for a variaty of acute and chronic liver diseases. Organ shortage, however, is becoming an increasing problem worldwide, limiting the applicability of liver transplantation. In addition, potential recipients are becoming sicker, thereby increasing the risk of loosing the graft during transplantation or in the initial post-operative period after liver transplantation (3 months). This trend is challenging the MELD allocation model where sickest candidates are prioritized and no delisting criteria are given...
November 10, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29129654/corrigendum-to-cause-of-death-in-patients-with-chronic-visceral-and-chronic-neurovisceral-acid-sphingomyelinase-deficiency-niemann-pick-disease-type-b-and-b-variant-literature-review-and-report-of-new-cases-mol-genet-metab-118-2016-206-213
#7
David Cassiman, Seymour Packman, Bruno Bembi, Hadhami Ben Turkia, Moeenaldeen Al-Sayed, Manuel Schiff, Jackie Imrie, Paulina Mabe, Tsutomu Takahashi, Karl Eugen Mengel, Roberto Giugliani, Gerald F Cox
No abstract text is available yet for this article.
November 9, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29119652/the-novel-language-systematic-aphasia-screening-saps-screening-based-therapy-in-combination-with-computerised-home-training
#8
Franziska Krzok, Verena Rieger, Katharina Niemann, Ruth Nobis-Bosch, Irmgard Radermacher, Walter Huber, Klaus Willmes, Stefanie Abel
BACKGROUND: SAPS-'Sprachsystematisches Aphasiescreening'-is a novel language-systematic aphasia screening developed for the German language, which already had been positively evaluated. It offers a fast assessment of modality-specific psycholinguistic components at different levels of complexity and the derivation of impairment-based treatment foci from the individual performance profile. However, SAPS has not yet been evaluated in combination with the new SAPS-based treatment. AIMS: To replicate the practicality of SAPS and to investigate the effectiveness of a SAPS-based face-to-face therapy combined with computerised home training in a feasibility study...
November 8, 2017: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/29119141/case-report-ursodeoxycholic-acid-treatment-in-niemann-pick-disease-type-c-clinical-experience-in-four-cases
#9
William R H Evans, Elena-Raluca Nicoli, Raymond Y Wang, Nina Movsesyan, Frances M Platt
In this case series, we demonstrate that Ursodeoxycholic acid (UDCA) improves liver dysfunction in Niemann-Pick type C (NPC) and may restore a suppressed cytochrome p450 system. NPC disease is a progressive neurodegenerative lysosomal storage disease caused by mutations in either the NPC1 or NPC2 genes. Liver disease is a common feature presenting either acutely as cholestatic jaundice in the neonatal period, or in later life as elevated liver enzymes indicative of liver dysfunction. Recently, an imbalance in bile acid synthesis in a mouse model of NPC disease was linked to suppression of the P450 detoxification system and was corrected by UDCA treatment...
2017: Wellcome Open Research
https://www.readbyqxmd.com/read/29116629/the-evolution-of-strain-typing-in-the-mycobacterium-tuberculosis-complex
#10
Matthias Merker, Thomas A Kohl, Stefan Niemann, Philip Supply
Tuberculosis (TB) is a contagious disease with a complex epidemiology. Therefore, molecular typing (genotyping) of Mycobacterium tuberculosis complex (MTBC) strains is of primary importance to effectively guide outbreak investigations, define transmission dynamics and assist global epidemiological surveillance of the disease. Large-scale genotyping is also needed to get better insights into the biological diversity and the evolution of the pathogen. Thanks to its shorter turnaround and simple numerical nomenclature system, mycobacterial interspersed repetitive unit-variable-number tandem repeat (MIRU-VNTR) typing, based on 24 standardized plus 4 hypervariable loci, optionally combined with spoligotyping, has replaced IS6110 DNA fingerprinting over the last decade as a gold standard among classical strain typing methods for many applications...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29100954/newborn-presentation-of-niemann-pick-disease-type-c-difficulties-and-limitations-of-diagnostic-methods
#11
Patryk Lipiński, Irena Jankowska, Agnieszka Ługowska, Małgorzata Musielak, Maciej Pronicki, Anna Tylki-Szymańska
No abstract text is available yet for this article.
October 12, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29090525/hematopoietic-stem-cell-transplantation-in-niemann-pick-disease-type-b-monitored-by-chitotriosidase-activity
#12
Paola Quarello, Spada Marco, Porta Francesco, Vassallo Elena, Timeus Fabio, Fagioli Franca
Here, we report a patient with Niemann-Pick disease type B, with early severe onset of disease and pulmonary involvement, treated with hematopoietic stem cell transplant (HSCT) from a bone marrow matched unrelated donor. We confirm that HSCT is feasible and potentially beneficial for patients with severe phenotype. Noteworthy, we discussed the potential usefulness of the activity of peripheral chitotriosidase for the longitudinal evaluation of HSCT success and effectiveness.
November 1, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29089554/reversible-tuning-of-magnetocaloric%C3%A2-ni-mn-ga-co-films-on-ferroelectric-pmn-pt-substrates
#13
Benjamin Schleicher, Robert Niemann, Stefan Schwabe, Ruben Hühne, Ludwig Schultz, Kornelius Nielsch, Sebastian Fähler
Tuning functional properties of thin caloric films by mechanical stress is currently of high interest. In particular, a controllable magnetisation or transition temperature is desired for improved usability in magnetocaloric devices. Here, we present results of epitaxial magnetocaloric Ni-Mn-Ga-Co thin films on ferroelectric Pb(Mg1/3Nb2/3)0.72Ti0.28O3 (PMN-PT) substrates. Utilizing X-ray diffraction measurements, we demonstrate that the strain induced in the substrate by application of an electric field can be transferred to the thin film, resulting in a change of the lattice parameters...
October 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29084218/concerted-regulation-of-npc2-binding-to-endosomal-lysosomal-membranes-by-bis-monoacylglycero-phosphate-and-sphingomyelin
#14
Giray Enkavi, Heikki Mikkolainen, Burçin Güngör, Elina Ikonen, Ilpo Vattulainen
Niemann-Pick Protein C2 (npc2) is a small soluble protein critical for cholesterol transport within and from the lysosome and the late endosome. Intriguingly, npc2-mediated cholesterol transport has been shown to be modulated by lipids, yet the molecular mechanism of npc2-membrane interactions has remained elusive. Here, based on an extensive set of atomistic simulations and free energy calculations, we clarify the mechanism and energetics of npc2-membrane binding and characterize the roles of physiologically relevant key lipids associated with the binding process...
October 2017: PLoS Computational Biology
https://www.readbyqxmd.com/read/29080444/murine-pluripotent-stem-cells-with-a-homozygous-knockout-of-foxg1-show-reduced-differentiation-towards-cortical-progenitors-in-vitro
#15
Eva Maria Mall, Doris Herrmann, Heiner Niemann
Foxg1 is a transcription factor critical for the development of the mammalian telencephalon. Foxg1 controls the proliferation of dorsal telencephalon progenitors and the specification of the ventral telencephalon. Homozygous knockout of Foxg1 in mice leads to severe microcephaly, attributed to premature differentiation of telencephalic progenitors, mainly of cortical progenitors. Here, we analyzed the influence of a Foxg1 knockout on differentiation of murine pluripotent stem cells (mPSCs) in an in vitro model of neuronal development...
October 19, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29079454/the-adenosine-a2a-receptor-agonist-t1-11-ameliorates-neurovisceral-symptoms-and-extends-the-lifespan-of-a-mouse-model-of-niemann-pick-type-c-disease
#16
Antonella Ferrante, Antonella Pezzola, Andrea Matteucci, Antonella Di Biase, Lucilla Attorri, Monica Armida, Alberto Martire, Yijuang Chern, Patrizia Popoli
Niemann-Pick C is a fatal neurovisceral disorder caused, in 95% of cases, by mutation of NPC1 gene. Therapeutic options are extremely limited and new "druggable" targets are highly warranted. We previously demonstrated that the stimulation of the adenosine A2A receptor (A2AR) normalized the pathological phenotype of cellular models of NPC1. Since the validation of A2ARs as a therapeutic target for NPC1 can be obtained only conducting studies in in vivo models of the disease, in the present paper, the effects of two agonists of A2ARs were evaluated in the mouse model Balb/c Npc1(nih), hereafter indicated as NPC1-/-...
October 25, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/29078232/mechanisms-involved-in-premature-aging-in-the-heart-is-there-an-implication-for-cardiac-surgery
#17
Bernd Niemann, Andreas Simm, Susanne Rohrbach
No abstract text is available yet for this article.
October 27, 2017: Thoracic and Cardiovascular Surgeon
https://www.readbyqxmd.com/read/29075811/-what-potential-do-geographic-information-systems-have-for-population-wide-health-monitoring-in-germany-perspectives-and-challenges-for-the-health-monitoring-of-the-robert-koch-institute
#18
REVIEW
Martin Thißen, Hildegard Niemann, Gianni Varnaccia, Alexander Rommel, Andrea Teti, Hans Butschalowsky, Kristin Manz, Jonas David Finger, Lars Eric Kroll, Thomas Ziese
Geographic information systems (GISs) are computer-based systems with which geographical data can be recorded, stored, managed, analyzed, visualized and provided. In recent years, they have become an integral part of public health research. They offer a broad range of analysis tools, which enable innovative solutions for health-related research questions. An analysis of nationwide studies that applied geographic information systems underlines the potential this instrument bears for health monitoring in Germany...
October 26, 2017: Bundesgesundheitsblatt, Gesundheitsforschung, Gesundheitsschutz
https://www.readbyqxmd.com/read/29074317/alteration-of-cortical-excitability-and-its-modulation-by-miglustat-in-niemann-pick-disease-type-c
#19
Shady Safwat Hassan, Carlos Trenado, Saskia Elben, Alfons Schnitzler, Stefan Jun Groiss
Niemann-Pick type C (NP-C) is a rare, neurodegenerative, lysosomal storage disease. Cortical excitability using different transcranial magnetic stimulation (TMS) protocols together with clinical and neuropsychological testing was longitudinally assessed in a patient with NP-C. Cerebellar inhibition, a measure for the integrity of the cerebello-thalamo-cortical network, was impaired. Short-latency afferent inhibition, a measure for cholinergic transmission, and cognitive functions were also impaired and improved under Miglustat treatment...
October 23, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29065835/hydroxypropyl-%C3%AE-cyclodextrin-formulated-in-nasal-chitosan-microspheres-as-candidate-therapeutic-agent-in-alzheimer-s-disease
#20
Giovanna Rassu, Elisabetta Gavini, Antonio Carta, Antonella Obinu, Elena Piera Porcu, Paolo Giunchedi
Hydroxypropyl-β-cyclodextrin (HP-CD) is a hydroxyalkyl derivative of native β-cyclodextrin, cyclic oligosaccharide able to form inclusion complexes with many drugs and biomolecules [1]. Cyclodextrins have a possible neuroprotective effect due to their capability to extract and deplete cholesterol from cell membranes [1]. HP-CD is classified as an excipient (cyclodextrins are in fact widely used to improve solubility of poorly water soluble drugs) and it is not been FDA-approved as therapeutic agent. However HP-CD received the "orphan drug designation" in the United States and Europe because it is employed in individual compassionate use for patients suffering from Niemann-Pick type C...
October 19, 2017: Current Drug Delivery
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