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https://www.readbyqxmd.com/read/28538051/mobius-syndrome-a-35-year-single-institution-experience
#1
Philip K McClure, Eray Kilinc, Scott Oishi, Anthony I Riccio, Lori A Karol
BACKGROUND: Mobius syndrome is a rare syndrome that is known to be associated with a variety of orthopaedic conditions including scoliosis, clubfoot, transverse limb deficiencies, Poland syndrome, and a myriad of hand conditions. To date, no large series exist to characterize the orthopaedic manifestations of Mobius syndrome. METHODS: Medical records at a single tertiary pediatric institution were reviewed for all patients diagnosed with Mobius syndrome from January 1, 1980 to December 31, 2015...
May 19, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28534340/imaging-of-cranial-nerves-iii-iv-vi-in-congenital-cranial-dysinnervation-disorders
#2
REVIEW
Jae Hyoung Kim, Jeong Min Hwang
Congenital cranial dysinnervation disorders are a group of diseases caused by abnormal development of cranial nerve nuclei or their axonal connections, resulting in aberrant innervation of the ocular and facial musculature. Its diagnosis could be facilitated by the development of high resolution thin-section magnetic resonance imaging. The purpose of this review is to describe the method to visualize cranial nerves III, IV, and VI and to present the imaging findings of congenital cranial dysinnervation disorders including congenital oculomotor nerve palsy, congenital trochlear nerve palsy, Duane retraction syndrome, Möbius syndrome, congenital fibrosis of the extraocular muscles, synergistic divergence, and synergistic convergence...
May 12, 2017: Korean Journal of Ophthalmology: KJO
https://www.readbyqxmd.com/read/28513664/a-resilient-and-luminescent-stimuli-responsive-hydrogel-from-a-heterotopic-1-8-naphthalimide-derived-ligand
#3
Chris S Hawes, Amy D Lynes, Kevin Byrne, Wolfgang Schmitt, Gavin Ryan, Matthias E Möbius, Thorfinnur Gunnlaugsson
A heterotopic naphthalimide ligand N-(4-picolyl)-4-(4'-carboxyphenoxy)-1,8-naphthalimide HL is utilised for the formation of self-assembled soft materials. In the presence of K(+) ions, L(-) forms a robust photoluminescent hydrogel 1 which is reversible under thermal, mechanical or chemical stimuli.
May 17, 2017: Chemical Communications: Chem Comm
https://www.readbyqxmd.com/read/28484846/erratum-to-antibody-mediated-neutralization-of-myelin-associated-ephrinb3-accelerates-cns-remyelination
#4
Yasir A Syed, Chao Zhao, Don Mahad, Wiebke Möbius, Friedrich Altmann, Franziska Foss, G A González, Aycan Sentürk, Amparo Acker-Palmer, Gert Lubec, Kathryn Lilley, Robin J M Franklin, Klaus-A Nave, Mark R N Kotter
No abstract text is available yet for this article.
May 8, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28479114/bronchopulmonary-dysplasia-where-have-all-the-stem-cells-gone-origin-and-potential-function-of-resident-lung-stem-cells
#5
Marius Alexander Möbius, Bernard Thébaud
Celebrating its 50(th) anniversary in 2017, bronchopulmonary dysplasia (BPD) - the chronic lung disease of prematurity that follows ventilator and O2 therapy for acute respiratory failure - remains the most frequent complication of extreme prematurity. Survival of premature infants born at increasingly earlier stages of gestation has made the prevention of lung injury increasingly challenging. BPD is postulated to be a misdirection of many functions in the developing lung including growth factor signalling and matrix as well as cellular composition, resulting in impaired alveolar and lung vascular growth...
May 4, 2017: Chest
https://www.readbyqxmd.com/read/28470148/peroxisomal-dysfunctions-cause-lysosomal-storage-and-axonal-kv1-channel-redistribution-in-peripheral-neuropathy
#6
Sandra Kleinecke, Sarah Richert, Livia de Hoz, Britta Brügger, Theresa Kungl, Ebrahim Asadollahi, Susanne Quintes, Judith Blanz, Rhona McGonigal, Kobra Naseri, Michael W Sereda, Timo Sachsenheimer, Christian Lüchtenborg, Wiebke Möbius, Hugh Willison, Myriam Baes, Klaus-Armin Nave, Celia Michèle Kassmann
Impairment of peripheral nerve function is frequent in neurometabolic diseases, but mechanistically not well understood. Here, we report a novel disease mechanism and the finding that glial lipid metabolism is critical for axon function, independent of myelin itself. Surprisingly, nerves of Schwann cell-specific Pex5 mutant mice were unaltered regarding axon numbers, axonal calibers, and myelin sheath thickness by electron microscopy. In search for a molecular mechanism, we revealed enhanced abundance and internodal expression of axonal membrane proteins normally restricted to juxtaparanodal lipid-rafts...
May 4, 2017: ELife
https://www.readbyqxmd.com/read/28446768/linear-dynamics-of-classical-spin-as-m%C3%A3-bius-transformation
#7
Alexey Galda, Valerii М Vinokur
Though the overwhelming majority of natural processes occur far from the equilibrium, general theoretical approaches to non-equilibrium phase transitions remain scarce. Recent breakthroughs introduced a description of open dissipative systems in terms of non-Hermitian quantum mechanics enabling the identification of a class of non-equilibrium phase transitions associated with the loss of combined parity (reflection) and time-reversal symmetries. Here we report that the time evolution of a single classical spin (e...
April 26, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28436155/the-mobius-airo-mobile-ct-for-image-guided-proton-therapy-characterization-commissioning
#8
Jasmine A Oliver, Omar A Zeidan, Sanford L Meeks, Amish P Shah, Jason Pukala, Patrick Kelly, Naren R Ramakrishna, Twyla R Willoughby
PURPOSE: The purpose of this study was to characterize the Mobius AIRO Mobile CT System for localization and image-guided proton therapy. This is the first known application of the AIRO for proton therapy. METHODS: Five CT images of a Catphan(®) 504 phantom were acquired on the AIRO Mobile CT System, Varian EDGE radiosurgery system cone beam CT (CBCT), Philips Brilliance Big Bore 16 slice CT simulator, and Siemens SOMATOM Definition AS 20 slice CT simulator. DoseLAB software v...
April 24, 2017: Journal of Applied Clinical Medical Physics
https://www.readbyqxmd.com/read/28422748/therapeutic-inhibition-of-soluble-brain-tnf-promotes-remyelination-by-increasing-myelin-phagocytosis-by-microglia
#9
Maria Karamita, Christopher Barnum, Wiebke Möbius, Malú G Tansey, David E Szymkowski, Hans Lassmann, Lesley Probert
Multiple sclerosis (MS) is an inflammatory CNS demyelinating disease in which remyelination largely fails. Transmembrane TNF (tmTNF) and TNF receptor 2 are important for remyelination in experimental MS models, but it is unknown whether soluble TNF (solTNF), a major proinflammatory factor, is involved in regeneration processes. Here, we investigated the specific contribution of solTNF to demyelination and remyelination in the cuprizone model. Treatment with XPro1595, a selective inhibitor of solTNF that crosses the intact blood-brain barrier (BBB), in cuprizone-fed mice did not prevent toxin-induced oligodendrocyte loss and demyelination, but it permitted profound early remyelination due to improved phagocytosis of myelin debris by CNS macrophages and prevented disease-associated decline in motor performance...
April 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28414271/monitoring-atp-dynamics-in-electrically-active-white-matter-tracts
#10
Andrea Trevisiol, Aiman S Saab, Ulrike Winkler, Grit Marx, Hiromi Imamura, Wiebke Möbius, Kathrin Kusch, Klaus-Armin Nave, Johannes Hirrlinger
In several neurodegenerative diseases and myelin disorders, the degeneration profiles of myelinated axons are compatible with underlying energy deficits. However, it is presently impossible to measure selectively axonal ATP levels in the electrically active nervous system. We combined transgenic expression of an ATP-sensor in neurons of mice with confocal FRET imaging and electrophysiological recordings of acutely isolated optic nerves. This allowed us to monitor dynamic changes and activity-dependent axonal ATP homeostasis at the cellular level and in real time...
April 17, 2017: ELife
https://www.readbyqxmd.com/read/28413599/rare-features-associated-with-mobius-syndrome-report-of-two-cases
#11
Rumela Ghosh, Vikram Shetty, Shruthi Hegde, G Subhas Babu, Vidya Ajila, Nanda Kishore P, Mithula Nair
Mobius syndrome is a rare congenital disorder with the preliminary diagnostic criteria of congenital facial and abducent nerve palsy. Involvement of other cranial nerves, too, is common. Prevalence rate of this syndrome is approximately 1 in 100,000 neonates. It is of unknown etiology with sporadic occurrence. However, data regarding the occurrence rate in India is limited. Features such as orofacial malformations, limb defects, and musculoskeletal, behavioral, and cognitive abnormalities might be associated...
2017: Journal of Dental Research, Dental Clinics, Dental Prospects
https://www.readbyqxmd.com/read/28401804/human-umbilical-cord-mesenchymal-stromal-cells-improve-survival-and-bacterial-clearance-in-neonatal-sepsis-in-rats
#12
Yueniu Zhu, Liqun Xu, Jennifer J P Collins, Arul Vadivel, Chanèle Cyr-Depauw, Shumei Zhong, Lars Mense, Marius A Möbius, Bernard Thébaud
Sepsis is the main cause of morbidity and mortality in neonates. Mesenchymal stromal cells (MSCs) are potent immune-modulatory cells. Their effect in neonatal sepsis has never been explored. We hypothesized that human umbilical cord-derived MSCs (hUC-MSCs) improve survival in experimental neonatal sepsis. Sepsis was induced in 3-day-old rats by intravenous injection of Escherichia coli (5 × 10(5)/rat). One hour after infection, rats were treated intravenously with normal saline, hUC-MSCs, or with interferon-γ preconditioned hUC-MSCs (10(7) cells/kg)...
May 17, 2017: Stem Cells and Development
https://www.readbyqxmd.com/read/28345573/clinical-correlation-of-imaging-findings-in-congenital-cranial-dysinnervation-disorders-involving-abducens-nerve
#13
Chanchal Gupta, Pradeep Sharma, Rohit Saxena, Ajay Garg, Sanjay Sharma
PURPOSE: High-resolution magnetic resonance imaging (MRI) of intracranial parts of sixth nerve and seventh nerve and the extraocular muscles (EOMs) in orbit to correlate the clinical characteristics in patients with two special forms of strabismus in congenital cranial dysinnervation disorders which are Duane's retraction syndrome (DRS) and Mobius syndrome. MATERIALS AND METHODS: Morphological analysis by 3T MRI of orbit (using surface coils) and brain (using 32 channel head coil) was performed on 6 patients with clinical DRS (1 bilateral), 2 cases with Mobius syndrome, and 1 case with congenital sixth nerve palsy...
February 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28337123/analysis-of-the-serotonergic-system-in-a-mouse-model-of-rett-syndrome-reveals-unusual-upregulation-of-serotonin-receptor-5b
#14
Steffen Vogelgesang, Sabine Niebert, Ute Renner, Wiebke Möbius, Swen Hülsmann, Till Manzke, Marcus Niebert
Mutations in the transcription factor methyl-CpG-binding-protein 2 (MeCP2) cause a delayed-onset neurodevelopmental disorder known as Rett syndrome (RTT). Although alteration in serotonin levels have been reported in RTT patients, the molecular mechanisms underlying these defects are not well understood. Therefore, we chose to investigate the serotonergic system in hippocampus and brainstem of male Mecp2(-/y) knock-out mice in the B6.129P2(C)-Mecp2(tm1.1Bird) mouse model of RTT. The serotonergic system in mouse is comprised of 16 genes, whose mRNA expression profile was analyzed by quantitative RT-PCR...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28295698/metallapentalenofurans-and-lactone-fused-metallapentalynes
#15
Zhengyu Lu, Congqing Zhu, Yuanting Cai, Jun Zhu, Yuhui Hua, Zhixin Chen, Jiangxi Chen, Haiping Xia
Metalla-aromatics are attractive species because they exhibit the properties of both organometallics and aromatics. Reported metal-bridged polycyclic aromatic complexes, as well as Möbius aromatic species, are still rare. Herein, we present the construction of two new metal-bridged polycyclic aromatic frameworks, α-metallapentalenofurans and lactone-fused metallapentalynes, by the reactions of osmapentalyne with terminal aryl alkynes in the presence of H2 O or HBF4 /H2 O, respectively. DFT calculations show that the α-metallapentalenofurans possess rare planar Craig-type Möbius aromaticity derived from the 11-center-12-electron dπ -pπ π-conjugation...
March 13, 2017: Chemistry: a European Journal
https://www.readbyqxmd.com/read/28291938/validation-of-a-modern-second-check-dosimetry-system-using-a-novel-verification-phantom
#16
Daniel G McDonald, Dustin J Jacqmin, Christopher J Mart, Nicholas C Koch, Jean L Peng, Michael S Ashenafi, Mario A Fugal, Kenneth N Vanek
PURPOSE: To evaluate the Mobius second-check dosimetry system by comparing it to ionization-chamber dose measurements collected in the recently released Mobius Verification Phantom™ (MVP). For reference, a comparison of these measurements to dose calculated in the primary treatment planning system (TPS), Varian Eclipse with the AcurosXB dose algorithm, is also provided. Finally, patient dose calculated in Mobius is compared directly to Eclipse to demonstrate typical expected results during clinical use of the Mobius system...
January 2017: Journal of Applied Clinical Medical Physics
https://www.readbyqxmd.com/read/28284609/evaluation-of-associations-between-genotypes-of-mycobacterium-avium-subsp-paratuberculsis-and-presence-of-intestinal-lesions-characteristic-of-paratuberculosis
#17
Petra Möbius, Elisabeth Liebler-Tenorio, Martin Hölzer, Heike Köhler
Mycobacterium avium subsp. paratuberculosis (MAP) is the causative agent of paratuberculosis affecting ruminants worldwide. Depending on the MAP-Type (MAP-C or MAP-S, cattle or sheep type), strains differ in virulence and host preference. There is not yet any strong evidence indicating that individual field strains of the same MAP-subgroup exhibit differences in virulence. The aim of this study was to evaluate a potential association between the genotype of individual field strains belonging to the MAP-C group and the presence of macroscopic intestinal lesions characteristic of paratuberculosis in the infected animals...
March 2017: Veterinary Microbiology
https://www.readbyqxmd.com/read/28254827/the-micos-component-mic60-displays-a-conserved-membrane-bending-activity-that-is-necessary-for-normal-cristae-morphology
#18
Daryna Tarasenko, Mariam Barbot, Daniel C Jans, Benjamin Kroppen, Boguslawa Sadowski, Gudrun Heim, Wiebke Möbius, Stefan Jakobs, Michael Meinecke
The inner membrane (IM) of mitochondria displays an intricate, highly folded architecture and can be divided into two domains: the inner boundary membrane adjacent to the outer membrane and invaginations toward the matrix, called cristae. Both domains are connected by narrow, tubular membrane segments called cristae junctions (CJs). The formation and maintenance of CJs is of vital importance for the organization of the mitochondrial IM and for mitochondrial and cellular physiology. The multisubunit mitochondrial contact site and cristae organizing system (MICOS) was found to be a major factor in CJ formation...
April 3, 2017: Journal of Cell Biology
https://www.readbyqxmd.com/read/28252243/internally-2-5-thienylene-bridged-46-decaphyrin-annuleno-annulene-network-consisting-of-m%C3%A3-bius-aromatic-thia-28-hexaphyrins-and-strong-h%C3%A3-ckel-aromaticity-of-its-protonated-form
#19
Takanori Soya, Hirotaka Mori, Yongseok Hong, Yun Hee Koo, Dongho Kim, Atsuhiro Osuka
Internally 1,3-phenylene- and 2,5-thienylene-bridged [46]decaphyrins 2 and 3 have been synthesized. While 2 shows modest aromatic character derived from the global 46π-conjugated circuit, 3 displays larger aromatic character owing to the contribution of an (annuleno)annulene-type network consisting of two twisted Möbius aromatic thia[28]hexaphyrin segments in addition to the global 46π-network. Upon protonation, these [46]decaphyrins underwent large structural changes to acquire strong aromaticity. Protonated 3 has been revealed to take on a planar structure composed of fused two triangular thia[28]hexaphyrin segments...
March 13, 2017: Angewandte Chemie
https://www.readbyqxmd.com/read/28248254/partial-immunoblotting-of-2d-gels-a-novel-method-to-identify-post-translationally-modified-proteins-exemplified-for-the-myelin-acetylome
#20
Kathrin Kusch, Marina Uecker, Thomas Liepold, Wiebke Möbius, Christian Hoffmann, Heinz Neumann, Hauke B Werner, Olaf Jahn
Post-translational modifications (PTMs) play a key role in regulating protein function, yet their identification is technically demanding. Here, we present a straightforward workflow to systematically identify post-translationally modified proteins based on two-dimensional gel electrophoresis. Upon colloidal Coomassie staining the proteins are partially transferred, and the investigated PTMs are immunodetected. This strategy allows tracking back the immunopositive antigens to the corresponding spots on the original gel, from which they are excised and mass spectrometrically identified...
January 12, 2017: Proteomes
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