bluuebunny/crossref_metadata_2025_split huggingface dataset:
Desmin prevents muscle wasting, exaggerated weakness and fragility, and fatigue in dystrophic mdx mouse
Authors: Ferry Arnaud , Messéant Julien , Parlakian Ara , Lemaitre Mégane , Roy Pauline , Delacroix Clément , Lilienbaum Alain , Hovhannisyan Yeranuhi , Furling Denis , Klein Arnaud , Li Zhenlin , Agbulut Onnik 
Published: June 2020

Key points
Desmin, similar to dystrophin, is associated with costameric structures bridging sarcomeres to the extracellular matrix.
Deletion of the desmin gene in mdx mice [double knockout (DKO) mice] induces marked muscle weakness and fatigue resistance compared to mdx mice.
Muscle fragility (higher susceptibility to contraction‐induced injury) was also aggravated in DKO mice compared to mdx mice.
By contrast to mdx mice, the DKO mice did not undergo muscle hypertrophy.
Desmin cDNA transfer with adeno‐associated virus in newborn mdx mice reduced muscle weakness.
Overall, desmin plays important and beneficial roles in muscle wasting, performance and fragility in dystrophic muscle.
AbstractDuchenne muscular dystrophy (DMD) is a severe neuromuscular disease caused by dystrophin deficiency. Desmin, similar to dystrophin, is associated with costameric structures bridging sarcomeres to the extracellular matrix that contributes to muscle function. In the present study, we attempted to provide further insight into the roles of desmin, for which the expression is increased in the muscle from the mouse mdx DMD model. We show that a deletion of the desmin gene (Des) in mdx mice [double knockout (DKO) mice, mdx:desmin–/–] induces a marked muscle weakness; namely, a reduced absolute maximal force production and increased fatigue compared to that in mdx mice. Fragility (i.e. higher susceptibility to contraction‐induced injury) was also aggravated in DKO mice compared to mdx mice, despite the promotion of supposedly less fragile muscle fibres in DKO mice, and this worsening of fragility was related to a decreased muscle excitability. Moreover, in contrast to mdx mice, the DKO mice did not undergo muscle hypertrophy, as indicated by smaller and fewer fibres, with a reduced percentage of centronucleated fibres, potentially explaining the severe muscle weakness. Notably, Desmin cDNA transfer with adeno‐associated virus in newborn mdx mice improved specific maximal force normalized to muscle weight. Overall, desmin plays important and beneficial roles in muscle wasting, performance and fragility in dystrophic mdx mice, which differ, at least in part, from those observed in healthy muscle.
Reconstruction of Trimmed NURBS Surfaces for Gap-Free Intersections
Authors: Urick Benjamin , Crawford Richard H. , Hughes Thomas J. R. , Cohen Elaine , Riesenfeld Richard F. 
Published: June 2020

Abstract
The modern engineering technologies of computer-aided design (CAD), computer-aided engineering (CAE), and computer-aided manufacturing (CAM) are ubiquitous in engineering practice. They are focused on creating, analyzing, and fabricating engineering artifacts represented as geometric models. Historically, these technologies developed independently, with different geometric representations that are customized to the needs of the technology. As a result, the combined use of these technologies has led to differences in data structures, file formats, and user knowledge and practice, requiring translation of representations between systems to support interoperability. Complicating this situation is the approximate nature of modeling operations in CAD systems, which can result in gaps at the boundary curves between mating trimmed surfaces of a model. The research presented here is aimed at removing the gaps between trimmed surfaces, resulting in a “watertight” model that is suitable for use directly by downstream applications. A three-step algorithm is presented that includes analysis of the parametric space of the trimming curves, reparameterization to create a global parameter space, and reconstruction of the intersecting surfaces to ensure continuity at the trimming curve.
Positioning Accuracy Reliability of Industrial Robots Through Probability and Evidence Theories
Authors: Zhang Dequan , Peng Zhouyuan , Ning Guosong , Han Xu 
Published: June 2020

Abstract
The positioning accuracy of industrial robots has an important influence on the stability and accuracy of robotic motion, which is one of the important indexes to measure the performance of robots. At present, some probability theory based methods are used to evaluate the positioning accuracy reliability of industrial robots. In practical engineering, the precise probability distribution of some robot’s parameters cannot be obtained directly. This study first uses the aleatory-epistemic hybrid model to describe the uncertain parameters of industrial robots. Second, the uncertain parameters are considered to construct the kinematic equation of industrial robots. Third, a probability-evidence hybrid reliability analysis model of industrial robots is established. Finally, the reliability interval of industrial robots under different thresholds can be obtained. Compared with the traditional method, the reliability results of industrial robots obtained by this method is an interval, which can more objectively evaluate the kinematics reliability of industrial robots. In the example, the effectiveness of the proposed method is verified by a six degrees of freedom (6-DoF) industrial robot.
Particle Swarm Optimization-Based Metaheuristic Design Generation of Non-Trivial Flat-Foldable Origami Tessellations With Degree-4 Vertices
Authors: Chen Yao , Yan Jiayi , Feng Jian , Sareh Pooya 
Published: June 2020

Abstract
Flat-foldable origami tessellations are periodic geometric designs that can be transformed from an initial configuration into a flat-folded state. There is growing interest in such tessellations, as they have inspired many innovations in various fields of science and engineering, including deployable structures, biomedical devices, robotics, and mechanical metamaterials. Although a range of origami design methods have been developed to generate such fold patterns, some non-trivial periodic variations involve geometric design challenges, the analytical solutions to which are too difficult. To enhance the design methods of such cases, this study first adopts a geometric-graph-theoretic representation of origami tessellations, where the flat-foldability constraints for the boundary vertices are considered. Subsequently, an optimization framework is proposed for developing flat-foldable origami patterns with four-fold (i.e., degree-4) vertices, where the boundaries of the unit fragment are given in advance. A metaheuristic using particle swarm optimization (PSO) is adopted for finding optimal solutions. Several origami patterns are studied to verify the feasibility and effectiveness of the proposed design method. It will be shown that in comparison with the analytical approach and genetic algorithms (GAs), the presented method can find both trivial and non-trivial flat-foldable solutions with considerably less effort and computational cost. Non-trivial flat-foldable patterns show different and interesting folding behaviors and enrich origami design.
An Experimental Investigation of Forces on Cortical Bone in Deep-Hole Bone Drilling
Authors: Lee JuEun , Chu Serena Y. 
Published: June 2020

Abstract
Deep-hole bone drilling is critical in many surgical implantation procedures. Unlike most common bone-drilling processes, deep-hole bone drilling is performed using a high drilling depth to drill-bit diameter ratio, which can lead to undesirable mechanical and thermal damage during surgical procedures. The objective of this study was to investigate the thrust force and torque generated in deep-hole bone drilling. Drilling tests were performed on bovine cortical bones at a drilling hole depth of 36 mm using a 2.5 mm diameter twist drill bit with a spindle speed of 3000 rpm and feed rates of 0.05, 0.075, and 0.1 mm/rev. Bone chips were collected at different depths and examined using a fiber-optic microscope. Not only are drilling forces a good indicator to assess drilling performances but also chip formation and morphology are important aspects for understanding bone-drilling behaviors. The force signals revealed two distinct states, which were referred to as normal and abnormal states in this study. In the normal state, the force signals remained constant once the drill tip became fully engaged in bone cutting, whereas after a certain drilling depth, the forces considerably increased in the abnormal state. The results of this study indicate that the rapid increase in the force in the abnormal state is mainly attributed to chip clogging inside the flutes as the drilling depth increases. This study also demonstrated that the chip morphology varies with respect to drilling depth, where fragmented chips are produced at shallow drilling depths and powdery chips are produced at deeper drilling depths.
Effect of Chemical Vapor Infiltration Induced Matrix Porosity on the Mechanical Behavior of Ceramic Matrix Minicomposites
Authors: Nagaraja Abhilash , Gururaja Suhasini 
Published: June 2020

Abstract
Ceramic matrix composites (CMCs) exhibit process-induced defects such as matrix porosity at multiple length scales that have a considerable influence on their mechanical and failure behavior. This work focuses on the microscale mechanical behavior of single tow CMCs in the presence of microporosities that exist within fiber bundles of the composite. Microporosities in a single tow C/boron nitride (BN)/SiC CMC minicomposite fabricated by chemical vapor infiltration (CVI) have been characterized by X-ray microcomputed tomography. The porosity distribution in the scanned region has been represented by probability distribution functions (PDFs) that serve as an input to numerical homogenization. Effective elastic properties in the presence of matrix micropores have been obtained by a two-step numerical homogenization approach considering the statistical distributions of pore parameters obtained from experimental characterization. A variation of the approach has been utilized to investigate the severity of pores with respect to their location and orientation relative to the fiber reinforcement.
Assessing Psychosocial Work Environments of Coaches in Spain and Their Relationships With Mental Health, Behavioral-Stress Symptoms, and Burnout
Authors: Hinojosa-Alcalde Ingrid , Andrés Ana , Didymus Faye F. , Norman Leanne , Soler Susanna 
Published: June 2020

The purpose of this study was to assess the psychosocial work environment (PWE) of a sample of coaches in comparison with the reference values of the Spanish general workforce, as well as to explore the relationship between PWE and mental health, behavioral-stress symptoms, and burnout. A representative sample (N = 1,481) of Spanish coaches (18.1% women, mean age = 32.98 years, SD = 11.60) completed a battery of questionnaires. Results showed that, in comparison with the general workforce, coaches showed statistically significant differences in most of the PWE areas assessed. The emotional demands experienced by coaches are a health risk, while trust regarding management and recognition are positive features in their PWE. Coaches’ emotional demands were associated with low mental-health scores and higher levels of behavioral-stress symptoms and burnout, whereas social community at work and role clarity were protective factors for health. Practical implications to provide more favorable work environments for coaches are discussed.
Late Devonian lonestones, diamictites, and coeval black shales from the Appalachian Basin: Discerning relationships and implications for Late Devonian Appalachian history and glacially driven seafloor anoxia
Authors: Ettensohn* Frank R. , Clayton Geoff , Lierman† R. Thomas , Mason Charles E. , Krause Federico F. , DeBuhr Christopher , Brackman Thomas B. , Anderson Eric D. , Dennis Allen J. , Pashin Jack C. 
Published: June 2020

ABSTRACT
A 3 ton (2.7 metric tonnes [t]), granitoid lonestone with Appalachian provenance was found in situ in offshore Devonian black shale in northeastern Kentucky, United States, and is denoted herein as the Robinson boulder, or lonestone, after its discoverer, Michael J. Robinson. This large boulder appears to have been displaced nearly 500 km from its source on the opposite margin of the Acadian/Neoacadian Appalachian foreland basin. While previous identifications of possible lonestones have been attributed to Pleistocene glacial events, scrutiny of this lonestone’s origin suggests that the boulder, which was embedded in the Upper Devonian Cleveland Shale Member of the Ohio Shale in northeastern Kentucky, is most likely a Devonian ice-rafted glacial dropstone. Notably, palynologic correlation with reported glacial diamictites elsewhere in the basin indicates such a source. Together, the dropstone and diamictites, separated by ~500 km, provide evidence for alpine glaciation in the ancient Acadian/Neoacadian orogen and for tidewater glaciers in the adjacent, eastern margin of the foreland basin. The latest Devonian marine transgression and Neoacadian foreland subsidence are interpreted to have been associated with tidewater glacial connections to the open sea. Importantly, the existence of this dropstone and its likely glacial precursor events require new considerations about contemporary black-shale sedimentation and the influence of tectonics on the delivery of glacial sediments to foreland basins.
Pliocene–Pleistocene landscape evolution and watershed reorganization east of the central Andes in Argentine Patagonia
Authors: Griffing Corinne Y. , Clague John J. , Barendregt Rene W. , Menounos Brian , Hemming Sidney R. , Rabassa Jorge , Ercolano Bettina , Martinez Oscar 
Published: June 2020

ABSTRACT
Uplift of the central Andes during the Miocene was followed by large-scale reorganization of Atlantic-draining rivers in Argentine Patagonia. Here, we document the abandonment of one large river in the late Pliocene and the establishment of the modern drainage in the Early Pleistocene. A chronology for these events is provided by 40Ar/39Ar ages on basalt flows. Remnants of the Pliocene paleovalley system are well preserved in the Lago Cardiel–Gobernador Gregores area, where they are eroded into flat-lying basalt flows dated from ca. 13.9 Ma to 8.6 Ma. Younger basalts that erupted onto the abandoned floor of the paleovalley are as young as 3.7 Ma. Abandonment of the Pliocene paleovalley and establishment of the modern Río Chico and Río Shehuen catchments happened near the close of the Pliocene when Andean glaciers incised the east-sloping pediment on which the late Miocene drainage was established. Lago Cardiel sits within a large endorheic basin that is inset into the late Pliocene paleovalley. The basin began to develop just before 4 Ma, after the paleovalley was abandoned. It became larger and deeper during the Pleistocene due to mass movements along its margins, deflation of the basin floor during times when Lago Cardiel was dry or nearly dry, and possibly lowering along bounding faults. The Pliocene–Pleistocene landscape and drainage changes that we have documented are not unique to the Lago Cardiel–Gobernador Gregores area; similar changes are apparent elsewhere in Patagonia east of the crest of the Andes.
Deglacial landforms and Holocene vegetation trajectories in the northern interior cedar-hemlock forests of British Columbia
Authors: Gavin* Daniel G. , White Ariana , Sanborn Paul T. , Hebda Richard J. 
Published: June 2020

ABSTRACT
The northern Rocky Mountain Trench of eastern British Columbia is a broad valley mantled by glaciolacustrine terraces supporting a complex mix of mesic-temperate (“interior wet belt”) forests that are strongly affected by terrain and substrate. Neither the geomorphic history during early Holocene deglaciation nor the vegetation history of the origin of the Tsuga heterophylla (western hemlock) and Thuja plicata (western redcedar) populations in the interior wet-belt forest is well understood. Sediment cores were obtained from two lakes, 10 km apart and occupying different terraces (83 m elevational difference), and these were compared to existing fire-history and paleoclimate reconstructions. Radiocarbon dates and a mapped terrain classification indicate the upper terrace formed as a lacustrine and glaciofluvial kame terrace hundreds of years prior to the lower terrace, which was formed by glaciolacustrine sediments of a proglacial lake. The minimum limiting ages of these terraces correlate with dated jökulhlaup deposits of the Fraser River. The upper site’s first detectable pollen at &gt;11.0 ka was dominated by light-seeded pioneer taxa (Poaceae [grasses], Artemisia [sagebrush], and Populus [aspen]) followed by a peak in Pinus (pine) and finally dominance by Betula (birch) at 10.2 ka. Pollen data suggest an earlier invasion of T. heterophylla (western hemlock) (by ca. 8 ka) than previously understood. Wetlands on extensive, poorly drained, glaciolacustrine soils promoted the persistence of boreal taxa and open forests (e.g., Picea mariana [black spruce]), while the better-drained upper kame terrace promoted development of closed-canopy shade-tolerant taxa. Invasion and expansion of mesic cedar-hemlock taxa progressed since at least the middle Holocene but was highly constrained by edaphic controls.
New paleontological evidence for complex middle Paleozoic tectonic evolution in the Appalachian western Blue Ridge
Authors: Lupo Mary E. , Tull James F. , Repetski John , Mueller Paul A. 
Published: June 2020

AbstractReconstructing the tectonic evolution of the southern Appalachian metamorphic internides is hampered by the relative paucity of accurate geochronologic constraints and the apparent rarity or absence of Paleozoic cover sequences. At the orogen’s greatest width, near the junction of Georgia, North Carolina, and Tennessee, the western Blue Ridge is a composite metamorphic allochthon of three major thrust sheets: (A) a basal sheet above the Great Smoky fault overlying rocks of the foreland thrust belt composed of the Lower Cambrian Chilhowee Group and underlying Sandsuck Formation of the Neoproterozoic Walden Creek Group; (B) an intermediate sheet above the Maggies Mill–Citico fault composed of the middle Paleozoic Maggies Mill Formation; and (C) the main mass of the western Blue Ridge above the Alaculsy Valley–Miller Cove fault composed of the Neoproterozoic Ocoee Supergroup, and younger overlying sequences in the Epperson and Murphy synclinoria. The age of peak deformation and metamorphism in all of these sequences has historically been assigned to the Ordovician Taconic orogeny, but recent paleontologic discoveries suggest these events are significantly younger. In addition to the middle Paleozoic fauna recently reported from the Maggies Mill Formation in the intermediate thrust sheet, Silurian-Devonian conodonts have been found in units formerly correlated with the Walden Creek Group in the Epperson synclinorium. These discoveries suggest that widespread middle Paleozoic successor basin sequences unconformably overlie the Neoproterozoic-Cambrian drift-facies of the Chilhowee Group (and equivalents) and underlying rift-facies of the Ocoee Supergroup, and require modifications to existing models for the timing of the region’s stratigraphic framework and tectono-metamorphic evolution.
Cyclostratigraphic calibration of the Eifelian Stage (Middle Devonian, Appalachian Basin, Western New York, USA)
Authors: Pas Damien , Da Silva Anne-Christine , Over D. Jeffrey , Brett Carlton E. , Brandt Lauren , Over Jin-Si , Hilgen Frederik J. , Dekkers Mark J. 
Published: June 2020

Abstract
Over the past decade the integration of astrochronology and U/Pb thermal ionization mass spectrometry dating has resulted in major improvements in the Devonian time scale, which allowed for accurate determination of ages and rates of change in this critical interval of Earth history. However, widely different durations have been published for the Middle Devonian Eifelian stage. Here we aim to solve this discrepancy by building an astronomically calibrated time scale using a high-resolution geochemical data set collected in the early to late Eifelian outer-ramp and deep-shelf deposits of the Seneca section (Appalachian Basin, Western New York, USA). The Middle Devonian Eifelian Stage (GTS2012; base at 393.3 ± 1.2 m.y. and duration estimate of 5.6 ± 1.9 m.y.), is bracketed by two major bioevents, respectively the Choteč event at its base and the Kačák event just prior to the Eifelian–Givetian boundary. To capture the record of Milankovitch-scale climatic cycles and to develop a model of the climatic and oceanographic variations that affected the Appalachian Basin during the Eifelian, 750 samples were collected at typically 2.5 cm intervals across the Seneca section. Major and trace elements were measured on each sample with an inductively coupled plasma–optical emission spectrometer. To estimate the duration of the Seneca section sampled, we applied multiple spectral techniques such as harmonic analysis, the multi-taper, and evolutionary spectral analysis, and we tuned the Log10Ti series using the short orbital eccentricity ∼100 k.y. cycle. Then, to assess the reliability of our cyclostratigraphic interpretation we ran the Average Spectral Misfit method on selected proxies for detrital input variation. The estimated duration derived using this method falls in the range of durations estimated with the tuning method. Using the approximate position of the Emsian–Eifelian and Eifelian–Givetian boundaries, constrained within &lt;1 m, the proposed estimation of the total duration of the Eifelian age is ∼5 m.y. Interpolated from the high-resolution U-Pb radiometric age available for the Tioga F Bentonite, the numerical ages of the Emsian–Eifelian and the Eifelian–Givetian were respectively recalibrated at 393.39 Ma and 388.24 Ma. The uncertainty from the radiometric date is respectively ± 0.86 Ma and ± 0.86 Ma.
Rare andde novoduplications containingSHOXin clubfoot
Authors: Sadler Brooke , Haller Gabe , Antunes Lilian , Nikolov Momchil , Amarillo Ina , Coe Bradley , Dobbs Matthew B. , Gurnett Christina A. 
Published: June 2020

IntroductionCongenital clubfoot is a common birth defect that affects at least 0.1% of all births. Nearly 25% cases are familial and the remaining are sporadic in inheritance. Copy number variants (CNVs) involving transcriptional regulators of limb development, includingPITX1andTBX4, have previously been shown to cause familial clubfoot, but much of the heritability remains unexplained.MethodsExome sequence data from 816 unrelated clubfoot cases and 2645 in-house controls were analysed using coverage data to identify rare CNVs. The precise size and location of duplications were then determined using high-density Affymetrix Cytoscan chromosomal microarray (CMA). Segregation in families andde novostatus were determined using qantitative PCR.ResultsChromosome Xp22.33 duplications involvingSHOXwere identified in 1.1% of cases (9/816) compared with 0.07% of in-house controls (2/2645) (p=7.98×10−5, OR=14.57) and 0.27% (38/13592) of Atherosclerosis Risk in Communities/the Wellcome Trust Case Control Consortium 2 controls (p=0.001, OR=3.97). CMA validation confirmed an overlapping 180.28 kb duplicated region that includedSHOXexons as well as downstream non-coding regions. In four of six sporadic cases where DNA was available for unaffected parents, the duplication wasde novo. The probability of fourde novomutations inSHOXby chance in a cohort of 450 sporadic clubfoot cases is 5.4×10–10.ConclusionsMicroduplications of the pseudoautosomal chromosome Xp22.33 region (PAR1) containingSHOXand downstream enhancer elements occur in ~1% of patients with clubfoot.SHOXand regulatory regions have previously been implicated in skeletal dysplasia as well as idiopathic short stature, but have not yet been reported in clubfoot.SHOXduplications likely contribute to clubfoot pathogenesis by altering early limb development.