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https://www.readbyqxmd.com/read/29336632/m%C3%A3-bius-syndrome-with-cardiac-rhabdomyomas
#1
Aimee Verner, Swati Agarwal-Sinha, Frank Yanfeng Han
BACKGROUND: Möbius syndrome is a rare congenital condition which presents not merely with 6th and 7th nerve palsies, but involves gaze paresis associated with craniofacial, limb, and other abnormalities. Heterogeneity is well known in patients with Möbius syndrome and rather than being of familial inheritance based on rare cases, it is much more recognized as a sporadic syndrome. We report an infant with features of congenital Möbius syndrome associated with cardiac rhabdomyomas in the absence of tuberous sclerosis...
January 16, 2018: Ophthalmic Genetics
https://www.readbyqxmd.com/read/29330215/mir-195-regulates-metabolism-in-failing-myocardium-via-alterations-in-sirt3-expression-and-mitochondrial-protein-acetylation
#2
Xiaokan Zhang, Ruiping Ji, Xianghai Liao, Estibaliz Castillero, Peter J Kennel, Danielle L Brunjes, Marcus Franz, Sven Möbius-Winkler, Konstantinos Drosatos, Isaac George, Emily I Chen, Paolo C Colombo, P Christian Schulze
Background -Heart failure (HF) leads to mitochondrial dysfunction and metabolic abnormalities of the failing myocardium coupled with an energy-depleted state and cardiac remodeling. The mitochondrial deacetylase sirtuin 3 (SIRT3) plays a pivotal role in the maintenance of mitochondrial function through regulating the mitochondrial acetylome. Interestingly, unique cardiac and systemic miRNAs have been shown to play an important role in cardiac remodeling by modulating key signaling elements in the myocardium...
January 12, 2018: Circulation
https://www.readbyqxmd.com/read/29325381/-longterm-surgery-outcome-of-m%C3%A3-bius-syndrome
#3
J Cen, X L Kang, J Yu, L Y Dong, Y Wei, K X Zhao
Objective: To investigate clinical features and the long-term surgery results of Möbius syndrome patients. Methods: This investigation presents a retrospective study of Möbius syndrome in 7 children we found since 2009. All 7 patients had MRI scan, ocular alignment in primary position, the limitation of versions and ductions and forced duction under general anesthesia. Early surgery is done to the 7 esotropia children. The pre-operative and post-operative outcomes, including the ocular alignment, deviation measurements and ocular rotations, were evaluated and compared...
December 11, 2017: [Zhonghua Yan Ke za Zhi] Chinese Journal of Ophthalmology
https://www.readbyqxmd.com/read/29323373/new-electron-delocalization-tools-to-describe-the-aromaticity-in-porphyrinoids
#4
Irene Casademont-Reig, Tatiana Woller, Julia Contreras-García, Mercedes Alonso, Miquel Torrent-Sucarrat, Eduard Matito
The role of aromaticity in porphyrinoids is a current subject of debate due to the intricate structure of these macrocycles, which can adopt Hückel, Möbius and even figure-eight conformers. One of the main challenges in these large π-conjugated structures is identifying the most conjugated pathway because, among aromaticity descriptors, there are very few that can be applied coherently to this variety of conformers. In this paper, we have investigated the conjugated pathways in nine porphyrinoid compounds using several aromaticity descriptors, including BLA, BOA, FLU and HOMA, as well as the recently introduced AV1245 and AVmin indices...
January 11, 2018: Physical Chemistry Chemical Physics: PCCP
https://www.readbyqxmd.com/read/29321813/novel-concept-of-a-modular-hip-implant-could-contribute-to-less-implant-failure-in-tha-a-hypothesis
#5
Ronny Grunert, Stefan Schleifenbaum, Robert Möbius, Michael Kopper, Christian Rotsch, Welf-Guntram Drossel, Niels Hammer, Torsten Prietzel
Background: The modularity in total hip arthroplasty (THA) allows orthopaedic surgeons for an exact reconstruction of hip biomechanical parameters especially in revision and tumor arthroplasty. Modular structured femoral stems using taper junctions showed increased implant breakage in the recent past. Presentation of the hypothesis: We hypothesize that a novel modular stem-neck-interface leads to less implant breakage compared to conventional femoral stems. Testing of the hypothesis: For this purpose, a novel modular femoral stem for THA was to design and manufacture...
2018: Patient Safety in Surgery
https://www.readbyqxmd.com/read/29301957/defective-cholesterol-clearance-limits-remyelination-in-the-aged-central-nervous-system
#6
Ludovico Cantuti-Castelvetri, Dirk Fitzner, Mar Bosch-Queralt, Marie-Theres Weil, Minhui Su, Paromita Sen, Torben Ruhwedel, Miso Mitkovski, George Trendelenburg, Dieter Lütjohann, Wiebke Möbius, Mikael Simons
Age-associated decline in regeneration capacity limits the restoration of nervous system functionality after injury. In a model for demyelination, we found that old mice fail to resolve the inflammatory response initiated after myelin damage. Aged phagocytes accumulated excessive amounts of myelin debris, which triggered cholesterol crystal formation, phagolysosomal membrane rupture, and stimulated inflammasomes. Myelin debris clearance required cholesterol transporters including apolipoprotein E. Remarkably, stimulation of reverse cholesterol transport was sufficient to restore the capacity of old mice to remyelinate lesioned tissue...
January 4, 2018: Science
https://www.readbyqxmd.com/read/29291371/conductance-switching-in-expanded-porphyrins-through-aromaticity-and-topology-changes
#7
Thijs Stuyver, Mickael L Perrin, Paul Geerlings, Frank De Proft, Mercedes Alonso
Expanded porphyrins are flexible enough to switch between different π-conjugation topologies, namely Mobius, Hückel and twisted-Hückel, each with distinct electronic properties and aromaticity. Since these switches can be induced by different external stimuli, expanded porphyrins represent a promising platform to develop a novel type of molecular switches for molecular electronic devices. In this work, the feasibility of conductance switches based on topology and/or aromaticity changes in expanded porphyrins is assessed for the first time...
January 1, 2018: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/29208351/detection-radius-of-emg-for-fasciculations-empiric-study-combining-ultrasonography-and-electromyography
#8
Martin Regensburger, Felix Tenner, Cornelia Möbius, Axel Schramm
OBJECTIVE: The aims of this study were to investigate the detection radius and sensitivity of EMG for fasciculations. METHODS: Muscle ultrasonography was performed simultaneously to EMG recordings in patients with fasciculations in the context of amyotrophic lateral sclerosis. Ultrasonography and EMG parameters were analyzed for selected fasciculations. RESULTS: A total of 381 fasciculations were detected by ultrasonography in 18 muscles of 10 patients...
November 21, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29195512/blood-brain-barrier-hyperpermeability-precedes-demyelination-in-the-cuprizone-model
#9
Stefan A Berghoff, Tim Düking, Lena Spieth, Jan Winchenbach, Sina K Stumpf, Nina Gerndt, Kathrin Kusch, Torben Ruhwedel, Wiebke Möbius, Gesine Saher
In neuroinflammatory disorders such as multiple sclerosis, the physiological function of the blood-brain barrier (BBB) is perturbed, particularly in demyelinating lesions and supposedly secondary to acute demyelinating pathology. Using the toxic non-inflammatory cuprizone model of demyelination, we demonstrate, however, that the onset of persistent BBB impairment precedes demyelination. In addition to a direct effect of cuprizone on endothelial cells, a plethora of inflammatory mediators, which are mainly of astroglial origin during the initial disease phase, likely contribute to the destabilization of endothelial barrier function in vivo...
December 1, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29194439/genetic-drift-and-selection-in-many-allele-range-expansions
#10
Bryan T Weinstein, Maxim O Lavrentovich, Wolfram Möbius, Andrew W Murray, David R Nelson
We experimentally and numerically investigate the evolutionary dynamics of four competing strains of E. coli with differing expansion velocities in radially expanding colonies. We compare experimental measurements of the average fraction, correlation functions between strains, and the relative rates of genetic domain wall annihilations and coalescences to simulations modeling the population as a one-dimensional ring of annihilating and coalescing random walkers with deterministic biases due to selection. The simulations reveal that the evolutionary dynamics can be collapsed onto master curves governed by three essential parameters: (1) an expansion length beyond which selection dominates over genetic drift; (2) a characteristic angular correlation describing the size of genetic domains; and (3) a dimensionless constant quantifying the interplay between a colony's curvature at the frontier and its selection length scale...
December 1, 2017: PLoS Computational Biology
https://www.readbyqxmd.com/read/29178860/a-new-classification-of-tka-periprosthetic-femur-fractures-considering-the-implant-type
#11
Johannes K M Fakler, Cathleen Pönick, Melanie Edel, Robert Möbius, Alexander Giselher Brand, Andreas Roth, Christoph Josten, Dirk Zajonz
BACKGROUND: The treatment aims of periprosthetic fractures (PPF) of the distal femur are a gentle stabilization, an early load-bearing capacity and a rapid postoperative mobilization of the affected patients. For the therapy planning of PPF a standardized classification is necessary which leads to a clear and safe therapy recommendation. Despite different established classifications, there is none that includes the types of prosthesis used in the assessment. For this purpose, the objective of this work is to create a new more extensive fracture and implant-related classification of periprosthetic fractures of the distal femur based on available classifications which allows distinct therapeutic recommendations...
November 25, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/29156061/bilateral-superior-rectus-transposition-with-bilateral-medial-rectus-recession-for-m%C3%A3-bius-syndrome
#12
Yuxi Zheng, Sean P Donahue
Bilateral superior rectus transposition with bilateral medial rectus recession has been described successfully for treatment of Duane's syndrome but never for Möbius syndrome. The authors describe a child with Möbius syndrome who presented with large bilateral abduction deficits and esotropia. This surgical treatment resulted in marked improvement of said deficits. [J Pediatr Ophthalmol Strabismus. 2017;54:e81-e82.].
November 17, 2017: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/29153254/facial-reanimation-surgery-in-m%C3%A3-bius-syndrome-experience-from-76-cases-from-a-tertiary-referral-hospital-in-latin-america
#13
A Cardenas-Mejia, D Palafox
INTRODUCTION: Möbius syndrome is defined as a combined congenital bilateral facial and abducens nerve palsies. The main goal of treatment is to provide facial reanimation by means of a dynamic surgical procedure. The microneurovascular transfer of a free muscle transplant is the procedure of choice for facial animation in a child with facial paralysis. OBSERVATION: Between January 2008 and January 2017, 124 patients with the syndrome have been approached at our institution...
November 15, 2017: Annales de Chirurgie Plastique et Esthétique
https://www.readbyqxmd.com/read/29151676/jim-hyde-and-the-endor-connection-a-personal-account
#14
Klaus Möbius, Wolfgang Lubitz, Anton Savitsky
In this minireview, we report on our year-long EPR work, such as electron-nuclear double resonance (ENDOR), pulse electron double resonance (PELDOR) and ELDOR-detected NMR (EDNMR) at X-band and W-band microwave frequencies and magnetic fields. This report is dedicated to James S. Hyde and honors his pioneering contributions to the measurement of spin interactions in large (bio)molecules. From these interactions, detailed information is revealed on structure and dynamics of macromolecules embedded in liquid-solution or solid-state environments...
2017: Applied Magnetic Resonance
https://www.readbyqxmd.com/read/29099393/filamentous-phages-as-building-blocks-for-reconfigurable-and-hierarchical-self-assembly
#15
Thomas Gibaud
Filamentous bacteriophages such as fd-like viruses are monodisperse rod-like colloids that have well defined properties: diameter, length, rigidity, charge and chirality. Engineering those viruses leads to a library of colloidal rods which can be used as building blocks for reconfigurable and hierarchical self-assembly. Their condensation in aqueous solution with additive polymers which act as depletants to induce attraction between the rods leads to a myriad of fluid-like micronic structures ranging from isotropic/nematic droplets, colloid membranes, achiral membrane seeds, twisted ribbons, $\pi$-wall, pores, colloidal skyrmions, Möbius anchors, scallop membranes to membrane rafts...
November 3, 2017: Journal of Physics. Condensed Matter: An Institute of Physics Journal
https://www.readbyqxmd.com/read/29098745/prosthodontic-management-of-a-patient-with-moebius-syndrome-a-clinical-report
#16
Ahmed Mahrous, Ghadeer Thalji
Moebius/Mӧbius Syndrome (MS) is an extremely rare congenital disorder characterized by uni- or bilateral palsy of the abducens (VI) and the facial (VII) nerves, causing facial paralysis. Dysfunction of cranial nerves III through XII is common, most often the glossopharyngeal (IX) and hypoglossus (XII). Afflicted individuals seeking prosthodontic care, particularly removable prosthetics, present multiple challenges related to inherent facial and tongue muscle weakness and microstomia. Other extraoral anomalies may include congenital multiple arthrogryposis and malformations of the upper and lower limbs...
November 3, 2017: Journal of Prosthodontics: Official Journal of the American College of Prosthodontists
https://www.readbyqxmd.com/read/29062251/a-congenital-cranial-dysinnervation-disorder-m%C3%A3-bius-syndrome
#17
Hatice Mutlu Albayrak, Nuriye Tarakçı, Hüseyin Altunhan, Rahmi Örs, Hüseyin Çaksen
Möbius' syndrome, also known as Möbius' sequence, is a nonprogressive cranial dysinnervation disorder characterized by congenital facial and abducens nerve paralysis. Here, we report a 5-day-old girl who was conceived after in vitro fertilization with poor suck and facial paralysis. She had bilaterally ptosis and lateral gaze limitation, left-sided deviation of the tongue, dysmorphic face, hypoplastic fingers and finger nails on the left hand, and was diagnosed as having Möbius' syndrome. Involvement of other cranial nerves such as three, four, five, nine, 9 and 12, and limb malformations may accompany this syndrome...
September 2017: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/29057888/multitwist-m%C3%A3-bius-strips-and-twisted-ribbons-in-the-polarization-of-paraxial-light-beams
#18
Enrique J Galvez, Ishir Dutta, Kory Beach, Jon J Zeosky, Joshua A Jones, Behzad Khajavi
The polarization of light can exhibit unusual features when singular optical beams are involved. In 3-dimensional polarized random media the polarization orientation around singularities describe 1/2 or 3/2 Möbius strips. It has been predicted that if singular beams intersect non-collinearly in free space, the polarization ellipse rotates forming many-turn Möbius strips or twisted ribbons along closed loops around a central singularity. These polarization features are important because polarization is an aspect of light that mediate strong interactions with matter, with potential for new applications...
October 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29047475/rainbows-by-elliptically-deformed-drops-ii-the-appearance-of-supernumeraries-of-high-order-rainbows-in-rain-showers
#19
Gunther P Können, James A Lock
The appearance of supernumeraries of high-order rainbows in heavy rain showers is explored for rainbows up to order five (p=6). This is done by using a combination of the ray-theory-based first-order Möbius approximation for high-order rainbows with the Airy approximation of the rainbow radiance distribution. We conclude that supernumerary formation of rainbows of order three, four, and five is possible in natural rain showers. Supernumeraries of the third-order and fourth-order rainbows are preferentially formed near the bottom of these rainbows...
July 1, 2017: Applied Optics
https://www.readbyqxmd.com/read/29047474/rainbows-by-elliptically-deformed-drops-i-m%C3%A3-bius-shift-for-high-order-rainbows
#20
James A Lock, Gunther P Können
Using ray theory, the Möbius shift of the (p-1)-order rainbow angle for a particle having an elliptical cross section is obtained to first order in the ellipticity as a function of the tilt of the ellipse with respect to the propagation direction of the incoming rays. The result is then adapted to the geometry of scattering of light rays from the sun by a falling water drop as a function of sun height angle. The variation in the angular spacing between the supernumeraries is determined as a function of location along the rainbow arc, the conditions under which the rainbow angle is insensitive to drop flattening were determined, and the dependence of the Möbius shift on the drop refractive index is shown for rainbows up to fourth order (p=5)...
July 1, 2017: Applied Optics
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