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https://www.readbyqxmd.com/read/28915917/translocation-of-molecular-chaperones-to-the-titin-springs-is-common-in-skeletal-myopathy-patients-and-affects-sarcomere-function
#1
Andreas Unger, Lisa Beckendorf, Pierre Böhme, Rudolf Kley, Marion von Frieling-Salewsky, Hanns Lochmüller, Rolf Schröder, Dieter O Fürst, Matthias Vorgerd, Wolfgang A Linke
Myopathies encompass a wide variety of acquired and hereditary disorders. The pathomechanisms include structural and functional changes affecting, e.g., myofiber metabolism and contractile properties. In this study, we observed increased passive tension (PT) of skinned myofibers from patients with myofibrillar myopathy (MFM) caused by FLNC mutations (MFM-filaminopathy) and limb-girdle muscular dystrophy type-2A due to CAPN3 mutations (LGMD2A), compared to healthy control myofibers. Because the giant protein titin determines myofiber PT, we measured its molecular size and the titin-to-myosin ratio, but found no differences between myopathies and controls...
September 15, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28912534/multisegmented-nanowires-a-step-towards-the-control-of-the-domain-wall-configuration
#2
E Berganza, M Jaafar, C Bran, J A Fernández-Roldán, O Chubykalo-Fesenko, M Vázquez, A Asenjo
Cylindrical nanowires synthesized by controlled electrodeposition constitute excellent strategic candidates to engineer magnetic domain configurations. In this work, multisegmented CoNi/Ni nanowires are synthesized for tailoring a periodic magnetic structure determined by the balance between magnetocrystalline and magnetostatic energies. High-resolution Transmission Electron Microscopy confirms the segmented growth and the sharp transition between layers. Although both CoNi and Ni segments have similar fcc cubic crystal symmetry, their magnetic configuration is quite different as experimentally revealed by Magnetic Force Microscopy (MFM) imaging...
September 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28888851/long-term-longitudinal-study-of-muscle-function-in-patients-with-glycogen-storage-disease-type-iiia
#3
Valérie Decostre, Pascal Laforêt, Marie De Antonio, Kahina Kachetel, Aurélie Canal, Gwenn Ollivier, Aleksandra Nadaj-Pakleza, François M Petit, Karim Wahbi, Abdallah Fayssoil, Bruno Eymard, Anthony Behin, Philippe Labrune, Jean-Yves Hogrel
Glycogen storage disease type III (GSDIII) is an autosomal recessive disorder caused by mutations in the AGL gene coding for the glycogen debranching enzyme. Current therapy is based on dietary adaptations but new preclinical therapies are emerging. The identification of outcome measures which are sensitive to disease progression becomes critical to assess the efficacy of new treatments in upcoming clinical trials. In order to prepare future longitudinal studies or therapeutic trials with large cohorts, information about disease progression is required...
August 30, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28858637/compensation-of-the-magnetic-force-imaging-by-scanning-directions
#4
Jinyun Liu, Miao Yu, Yingmin Qu, Wenxiao Zhang, Yinxue Fan, Zhengxun Song, Renxi Qiu, Dayou Li, Zuobin Wang
It was found that the results of magnetic force microscope (MFM) imaging were different with the probe scanning directions. This paper studied the effect of scanning directions on the MFM imaging, and a method for the distortion compensation was proposed to reduce the errors. In the study, three different scanning directions with the angles of 0°, 45° and 90° were used to measure the magnetic domain structures distributions of magnetic sample. The experimental results have shown that the scanning direction parallel to the magnetic domain structure will cause a minimum phase shift difference and lead to a structure distortion...
August 26, 2017: Micron: the International Research and Review Journal for Microscopy
https://www.readbyqxmd.com/read/28841698/long-term-follow-up-of-mri-changes-in-thigh-muscles-of-patients-with-facioscapulohumeral-dystrophy-a-quantitative-study
#5
Farzad Fatehi, Emmanuelle Salort-Campana, Arnaud Le Troter, Emilie Lareau-Trudel, Mark Bydder, Alexandre Fouré, Maxime Guye, David Bendahan, Shahram Attarian
Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common hereditary muscular disorders. Currently FSHD has no known effective treatment and detailed data on the natural history are lacking. Determination of the efficacy of a given therapeutic approach might be difficult in FSHD given the slow and highly variable disease progression. Magnetic resonance imaging (MRI) has been widely used to qualitatively and quantitatively evaluate in vivo the muscle alterations in various neuromuscular disorders...
2017: PloS One
https://www.readbyqxmd.com/read/28775275/calibration-of-multi-layered-probes-with-low-high-magnetic-moments
#6
Vishal Panchal, Héctor Corte-León, Boris Gribkov, Luis Alfredo Rodriguez, Etienne Snoeck, Alessandra Manzin, Enrico Simonetto, Silvia Vock, Volker Neu, Olga Kazakova
We present a comprehensive method for visualisation and quantification of the magnetic stray field of magnetic force microscopy (MFM) probes, applied to the particular case of custom-made multi-layered probes with controllable high/low magnetic moment states. The probes consist of two decoupled magnetic layers separated by a non-magnetic interlayer, which results in four stable magnetic states: ±ferromagnetic (FM) and ±antiferromagnetic (A-FM). Direct visualisation of the stray field surrounding the probe apex using electron holography convincingly demonstrates a striking difference in the spatial distribution and strength of the magnetic flux in FM and A-FM states...
August 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28772374/phase-equilibria-and-magnetic-phases-in-the-ce-fe-co-b-system
#7
Tian Wang, Dmytro Kevorkov, Mamoun Medraj
Ce-Fe-Co-B is a promising system for permanent magnets. A high-throughput screening method combining diffusion couples, key alloys, Scanning Electron Microscope/Wavelength Dispersive X-ray Spectroscope (SEM/WDS), and Magnetic Force Microscope (MFM) is used in this research to understand the phase equilibria and to explore promising magnetic phases in this system. Three magnetic phases were detected and their homogeneity ranges were determined at 900 °C, which were presented by the formulae: Ce₂Fe14-xCoxB (0 ≤ x ≤ 4...
December 28, 2016: Materials
https://www.readbyqxmd.com/read/28772068/porous-metal-organic-polyhedral-frameworks-with-optimal-molecular-dynamics-and-pore-geometry-for-methane-storage
#8
Yong Yan, Daniil I Kolokolov, Ivan da Silva, Alexander G Stepanov, Alexander J Blake, Anne Dailly, Pascal Manuel, Chiu C Tang, Sihai Yang, Martin Schröder
Natural gas (methane, CH4) is widely considered as a promising energy carrier for mobile applications. Maximising the storage capacity is the primary goal for the design of future storage media. Here we report the CH4 storage properties in a family of isostructural (3,24)-connected porous materials, MFM-112a, MFM-115a and MFM-132a with different linker backbone functionalisation. Both MFM-112a and MFM-115a show excellent CH4 uptakes of 236 and 256 cm3 (STP) cm-3 (v/v) at 80 bar and room temperature, respectively...
August 3, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28725270/inter-segmental-motions-of-the-foot-differences-between-younger-and-older-healthy-adult-females
#9
Dong Yeon Lee, Sang Gyo Seo, Eo Jin Kim, Doo Jae Lee, Kee Jeong Bae, Kyoung Min Lee, In Ho Choi
BACKGROUND: Although accumulative evidence exists that support the applicability of multi-segmental foot models (MFMs) in evaluating foot motion in various pathologic conditions, little is known of the effect of aging on inter-segmental foot motion. The objective of this study was to evaluate differences in inter-segmental motion of the foot between older and younger adult healthy females during gait using a MFM with 15-marker set. METHODS: One hundred symptom-free females, who had no radiographic evidence of osteoarthritis, were evaluated using MFM with 15-marker set...
2017: Journal of Foot and Ankle Research
https://www.readbyqxmd.com/read/28706216/highly-efficient-multicolor-multifocus-microscopy-by-optimal-design-of-diffraction-binary-gratings
#10
Bassam Hajj, Laura Oudjedi, Jean-Bernard Fiche, Maxime Dahan, Marcelo Nollmann
Multifocus microscopy (MFM) allows sensitive and fast three-dimensional imaging. It relies on the efficient design of diffraction phase gratings yielding homogeneous intensities in desired diffraction orders. Such performances are however guaranteed only for a specific wavelength. Here, we discuss a novel approach for designing binary phase gratings with dual color properties and improved diffraction efficiency for MFM. We simulate binary diffraction gratings with tunable phase shifts to explore its best diffraction performances...
July 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28705036/pregnancy-outcomes-among-women-with-systemic-lupus-erythematosus-a-retrospective-cohort-study-from-thailand
#11
S Phansenee, R Sekararithi, P Jatavan, T Tongsong
Objective The objective of this paper is to compare adverse pregnancy outcomes between normal pregnancies and pregnancies with systemic lupus erythematosus (SLE), particularly focusing on uncomplicated SLE with remission. Methods A retrospective cohort study was conducted by accessing the Maternal-Fetal Medicine (MFM) Unit database and the full medical records of the women. The records of singleton pregnancies with SLE and no underlying disease were assigned as the study group and their medical records were reviewed...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28651447/the-association-between-fetal-fibronectin-and-spontaneous-preterm-birth-in-twin-pregnancies-with-a-shortened-cervical-length
#12
Kathy C Matthews, Simi Gupta, Jennifer Lam-Rachlin, Daniel H Saltzman, Andrei Rebarber, Nathan S Fox
OBJECTIVE: To estimate the association between a positive fetal fibronectin (fFN) and spontaneous preterm birth (SPTB) in twin pregnancies with a shortened cervical length (CL). STUDY DESIGN: Retrospective cohort study of asymptomatic twin pregnancies managed by a single MFM practice from 2005 to 2016. We included all women with a shortened CL ≤25 mm at 22-28 weeks, and compared outcomes between women with a positive and negative fFN result. RESULTS: One hundred fifty-five patients were included, 129 (83...
July 10, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28629247/obstetric-practice-patterns-in-pregnancies-complicated-by-fetal-trisomy-13-or-18
#13
Sarah K Dotters-Katz, Marcela C Smid, Cora Mcelwain, Jeffrey A Kuller, Jay Schulkin
PURPOSE: Describe practice patterns among obstetrician/gynecologists (OB/GYNs) when caring for women with pregnancy complicated by fetal trisomy 13(T13) or 18(T18), and compare these between maternal-fetal-medicine (MFM) and non-MFM providers. MATERIALS AND METHODS: We conducted an electronic survey using the American College of Obstetricians and Gynecologists database. Using simple statistics, we describe demographics and practice patterns among respondents, and compare those of MFM practitioners with non-MFM providers...
June 19, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28622600/grain-size-quantification-by-optical-microscopy-electron-backscatter-diffraction-and-magnetic-force-microscopy
#14
Hansheng Chen, Yin Yao, Jacob A Warner, Jiangtao Qu, Fan Yun, Zhixiao Ye, Simon P Ringer, Rongkun Zheng
Quantification of microstructure, especially grain size, in polycrystalline materials is a vital aspect to understand the structure-property relationships in these materials. In this paper, representative characterization techniques for determining the grain size, including optical microscopy (OM), electron backscatter diffraction (EBSD) in the scanning electron microscopy (SEM), and atomic force microscopy/magnetic force microscopy (AFM/MFM), are thoroughly evaluated in comparison, illustrated by rare-earth sintered Nd-Fe-B permanent magnets...
June 13, 2017: Micron: the International Research and Review Journal for Microscopy
https://www.readbyqxmd.com/read/28620542/preconception-consultations-with-maternal-fetal-medicine-for-obese-women-a-retrospective-chart-review
#15
Charlotte M Page, Elizabeth S Ginsburg, Randi H Goldman, Chloe A Zera
BACKGROUND: Obesity is associated with impaired fertility and pregnancy complications, and preconception weight loss may improve some of these outcomes. The purpose of this study was to evaluate the quality and effectiveness of Maternal Fetal Medicine (MFM) preconception consults for obese women. METHODS: We performed a retrospective chart review examining 162 consults at an academic medical center from 2008 to 2014. The main outcome measures included consultation content - e...
2017: Fertility Research and Practice
https://www.readbyqxmd.com/read/28555689/epitaxial-magnetite-nanorods-with-enhanced-room-temperature-magnetic-anisotropy
#16
Sayan Chandra, Raja Das, Vijaysankar Kalappattil, Tatiana Eggers, Catalin Harnagea, Riad Nechache, Manh-Huong Phan, Federico Rosei, Hariharan Srikanth
Nanostructured magnetic materials with well-defined magnetic anisotropy are very promising as building blocks in spintronic devices that operate at room temperature. Here we demonstrate the epitaxial growth of highly oriented Fe3O4 nanorods on a SrTiO3 substrate by hydrothermal synthesis without the use of a seed layer. The epitaxial nanorods showed biaxial magnetic anisotropy with an order of magnitude difference between the anisotropy field values of the easy and hard axes. Using a combination of conventional magnetometry, transverse susceptibility, magnetic force microscopy (MFM) and magneto-optic Kerr effect (MOKE) measurements, we investigate magnetic behavior such as temperature dependent magnetization and anisotropy, along with room temperature magnetic domain formation and its switching...
May 30, 2017: Nanoscale
https://www.readbyqxmd.com/read/28543538/clinical-and-histopathological-features-of-myofibrillar-myopathy-in-warmblood-horses
#17
S J Valberg, A M Nicholson, S S Lewis, R A Reardon, C J Finno
BACKGROUND: To report a novel exertional myopathy, myofibrillar myopathy (MFM) in Warmblood (WB) horses. OBJECTIVES: To 1) describe the distinctive clinical and myopathic features of MFM in Warmblood horses and 2) investigate the potential inheritance of MFM in a Warmblood family. STUDY DESIGN: Retrospective selection of MFM cases and prospective evaluation of a Warmblood family. METHODS: Retrospectively, muscle biopsies were selected from Warmblood horses diagnosed with MFM and clinical histories obtained (n = 10)...
May 22, 2017: Equine Veterinary Journal
https://www.readbyqxmd.com/read/28523528/comparison-of-visual-outcomes-and-reading-performance-after-bilateral-implantation-of-multifocal-intraocular-lenses-with-bilateral-monofocal-intraocular-lenses
#18
Özge Güngör Akkuş, Ikbal Seza Petriçli
PURPOSE: To compare the visual acuity outcomes, contrast sensitivity function (CS) and reading ability in patients with bilateral multifocal intraocular lenses and patients with bilateral monofocal lenses. SETTING: Vizyon Eye Center, Denizli, Turkey. DESIGN: Comparative case series. METHODS: Consecutive bilateral cataract patients having implantation of Acriva Reviol MFM 611 multifocal IOLs (Group A) or Acriva BB UD 613 monofocal IOLs (Group B) were included...
May 18, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28507700/stepwise-observation-and-quantification-and-mixed-matrix-membrane-separation-of-co2-within-a-hydroxy-decorated-porous-host
#19
Christopher G Morris, Nicholas M Jacques, Harry G W Godfrey, Tamoghna Mitra, Detlev Fritsch, Zhenzhong Lu, Claire A Murray, Jonathan Potter, Tom M Cobb, Fajin Yuan, Chiu C Tang, Sihai Yang, Martin Schröder
The identification of preferred binding domains within a host structure provides important insights into the function of materials. State-of-the-art reports mostly focus on crystallographic studies of empty and single component guest-loaded host structures to determine the location of guests. However, measurements of material properties (e.g., adsorption and breakthrough of substrates) are usually performed for a wide range of pressure (guest coverage) and/or using multi-component gas mixtures. Here we report the development of a multifunctional gas dosing system for use in X-ray powder diffraction studies on Beamline I11 at Diamond Light Source...
April 1, 2017: Chemical Science
https://www.readbyqxmd.com/read/28506026/raman-submicron-spatial-mapping-of-individual-mn-doped-zno-nanorods
#20
V Strelchuk, O Kolomys, S Rarata, P Lytvyn, O Khyzhun, Chan Oeurn Chey, Omer Nur, Magnus Willander
ZnO nanorods (NRs) arrays doped with a large concentration of Mn synthesized by aqueous chemical growth and were characterized by SEM, photoluminescence, Raman scattering, magnetic force microscopy (MFM). By comparison of spectra taken on pure and Mn-doped ZnO NRs, a few new Raman impurity-related phonon modes, resulting from the presence of Mn in the investigated samples. We also present a vibrational and magnetic characterization of individual lying nanorods using Raman and MFM imaging. Confocal scanning micro-Raman mapping of the spatial distribution of intensity and frequency of phonon modes in single Mn-doped ZnO NRs nanorods is presented and analyzed for the first time...
December 2017: Nanoscale Research Letters
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