Lamin-Related Congenital Muscular Dystrophy Alters Mechanical Signaling and Skeletal Muscle Growth

Laminopathies are a clinically heterogeneous group of disorders caused by mutations in the LMNA gene, which encodes the nuclear envelope proteins lamins A and C. The most frequent diseases associated with LMNA mutations are characterized by skeletal and cardiac involvement, and include autosomal dominant Emery–Dreifuss muscular dystrophy (EDMD), limb-girdle muscular dystrophy type 1B, and LMNA-related congenital muscular dystrophy (LMNA-CMD). Although the exact pathophysiological mechanisms responsible for LMNA-CMD are not yet understood, severe contracture and muscle atrophy suggest that mutations may impair skeletal muscle growth. Using human muscle stem cells (MuSCs) carrying LMNA-CMD mutations, we observe impaired myogenic fusion with disorganized cadherin/β catenin adhesion complexes. We show that skeletal muscle from Lmna-CMD mice is unable to hypertrophy in response to functional overload, due to defective fusion of activated MuSCs, defective protein synthesis and defective remodeling of the neuromuscular junction. Moreover, stretched myotubes and overloaded muscle fibers with LMNA-CMD mutations display aberrant mechanical regulation of the yes-associated protein (YAP). We also observe defects in MuSC activation and YAP signaling in muscle biopsies from LMNA-CMD patients. These phenotypes are not recapitulated in closely related but less severe EDMD models. In conclusion, combining studies in vitro, in vivo, and patient samples, we find that LMNA-CMD mutations interfere with mechanosignaling pathways in skeletal muscle, implicating A-type lamins in the regulation of skeletal muscle growth.     

A Hybrid Virtual Cloud Learning Model during the COVID-19 Pandemic

The COVID-19 pandemic has affected the educational systems worldwide, leading to the near-total closures of schools, universities, and colleges. Universities need to adapt to changes to face this crisis without negatively affecting students’ performance. Accordingly, the purpose of this study is to identify and help solve to critical challenges and factors that influence the e-learning system for Computer Maintenance courses during the COVID-19 pandemic. The paper examines the effect of a hybrid modeling approach that uses Cloud Computing Services (CCS) and Virtual Reality (VR) in a Virtual Cloud Learning Environment (VCLE) system. The VCLE system provides students with various utilities and educational services such as presentation slides/text, data sharing, assignments, quizzes/tests, and chatrooms. In addition, learning through VR enables the students to simulate physical presence, and they respond well to VR environments that are closer to reality as they feel that they are an integral part of the environment. Also, the research presents a rubric assessment that the students can use to reflect on the skills they used during the course. The research findings offer useful suggestions for enabling students to become acquainted with the proposed system’s usage, especially during the COVID-19 pandemic, and for improving student achievement more than the traditional methods of learning.     

Study and characterization of porous copper oxide produced by electrochemical anodization for radiometric heat absorber

The aim of this work is to optimize the different parameters for realization of an absorbing cavity to measure the incident absolute laser energy. Electrochemical oxidation is the background process that allowed the copper blackening. A study of the blackened surface quality was undertaken using atomic force microscopy (AFM) analysis and ultraviolet-visible-infrared spectrophotometry using a Shimadzu spectrophotometer. A two-dimensional and three-dimensional visualization by AFM of the formed oxide coating showed that the copper surfaces became porous after electrochemical etching with different roughness. This aspect is becoming more and more important with decreasing current density anodization. In a 2 mol L ^-1 of NaOH solution, at a temperature of 90°C, and using a 16 mA cm² constant density current, the copper oxide formed has a reflectivity of around 3% in the spectral range between 300 and 1,800 nm. Using the ‘mirage effect’ technique, the obtained Cu₂O diffusivity and thermal conductivity are respectively equal to (11.5 ± 0.5) 10 to 7 m² s^-1 and (370 ± 20) Wm^-1 K^-1. This allows us to consider that our Cu₂O coating is a good thermal conductor. The results of the optical and thermal studies dictate the choice of the cavity design. The absorbing cavity is a hollow cylinder machined to its base at an angle of 30°. If the included angle of the plane is 30° and the interior surface gives specular reflection, an incoming ray parallel to the axis will undergo five reflections before exit. So the absorption of the surface becomes closely near 0.999999.     

Optimal Sizing of a DBD Ozone Generator Using Response Surface Modeling

Dielectric barrier discharge (DBD) reactors used as ozone generators are well known today and widely used for water treatment and air disinfection. The purpose of this article is to propose an experimental procedure based on the response surface modeling in order to optimize the geometrical dimensions of the cylindrical shape ozone generator, i.e., the discharge gap and the electrodes length. Because an effective ozone generator is expected to give high ozone concentration with a minimum of power requirements, the applied high voltage was associated with the geometrical parameters to carry out a composite centered faces design. Obtained results indicate that for an efficient ozone generator, length of the electrodes needs to be optimized while the discharge gap should be minimized.     

Mono‐, Di‐ and Tetra‐Cationic Surfactants as Carbon Steel Corrosion Inhibitors

Corrosion inhibition of three new synthesized cationic surfactants, N‐(2‐(((Z)‐4‐(pyridin‐4‐yl)but‐3‐en‐1‐yl)amino)ethyl)‐N‐(2‐((E)‐(pyridin‐4‐ylmethylene)amino)ethyl)dodecan‐1‐aminium bromide I(4N), N¹,N²‐didodecyl‐N¹‐((Z)‐4‐(pyridin‐4‐yl)but‐3‐en‐1‐yl)‐N²‐(2‐((E)‐(pyridin‐4‐ylmethylene)amino)ethyl)ethane‐1,2‐diaminium bromide II(4N) and 1‐dodecyl‐4‐((E)‐((2‐(dodecyl(2‐(dodecyl((Z)‐4‐(1‐dodecylpyridin‐1‐ium‐4‐yl)but‐3‐en‐1‐yl)ammonio)ethyl)ammonio)ethyl)imino)methyl)pyridin‐1‐ium bromide IV(4N) on carbon steel was investigated by weight loss, electrochemical impedance spectroscopy and polarization measurements. Results show that the synthesized cationic surfactants inhibit corrosion of carbon steel in 1 M HCl. The inhibitive action occurs by virtue of adsorption on the metal surface following a Langmuir adsorption isotherm model. Polarization curves reveal that the investigated cationic surfactants can be classified as mixed inhibitor types. The variations in the corrosion inhibition efficiency between three cationic surfactants are correlated with their chemical structures, with more hydrophobic surfactants yielding higher inhibition efficiency.     

A new interval diagnosis method: Application to the spacecraft rendezvous phase of the Mars sample return mission

This paper presents a new optimized interval principal component analysis applied to detect and isolate actuators faults of an autonomous spacecraft involved in the rendezvous phase of the Mars sample return mission. Based on the exploitation of various arithmetic and interval analysis properties, the new interval model is built by solving the interval eigenpairs problem via a resolution of a parametric linear programming problem. The detection and isolation phases are performed by extending the classic methods to interval-valued data. The proposed method is applied to detect and isolate actuators faults that can occur on the spacecraft's thrusters. Based on data provided by a “high fidelity” industrial simulator developed by Thales Alenia Space, the obtained results proved the effectiveness of the proposed interval fault diagnosis method on detecting and isolating thrusters' faults.     

New approach to mimic rheological actual shear rate under wall slip condition

Engineering with Computers - The presence of wall slip in concentrated suspensions affect the rheological measurements such as shear stress, shear rate, and viscosity. The measured shear rate will...     

The ETH‐MAV Team in the MBZ International Robotics Challenge

This study describes the hardware and software systems of the Micro Aerial Vehicle (MAV) platforms used by the ETH Zurich team in the 2017 Mohamed Bin Zayed International Robotics Challenge (MBZIRC). The aim was to develop robust outdoor platforms with the autonomous capabilities required for the competition, by applying and integrating knowledge from various fields, including computer vision, sensor fusion, optimal control, and probabilistic robotics. This paper presents the major components and structures of the system architectures and reports on experimental findings for the MAV‐based challenges in the competition. Main highlights include securing the second place both in the individual search, pick, and place the task of Challenge 3 and the Grand Challenge, with autonomous landing executed in less than 1 min and a visual servoing success rate of over for object pickups.     

The pleural thickening approximation from thoracic CT scans

Multimedia Tools and Applications - The involvement of medical imaging in medical procedures plays an important role in the diagnosis and planning of a therapeutic treatment. Computerized...