Length Expansion Research Articles

The role of NOTCH2NLC in Parkinson's disease: A clinical, neuroimaging, and pathological study.

Recently, the pathogenic and intermediate GGC repeat expansion in NOTCH2NLC was detected in Parkinson's disease (PD). However, detailed clinical, neuroimaging, and pathological information of clinically diagnosed PD patients with pathogenic GGC repeat expansion in NOTCH2NLC remains scarce. Thus, we aimed to elucidate the clinical, neuroimaging, and pathological characteristics of PD patients carrying the pathogenic GGC repeat expansion in NOTCH2NLC. The NOTCH2NLC GGC repeat expansion was screened in 941 sporadic PD patients and 244 unrelated probands. Comprehensive assessments were performed in three PD patients with pathogenic GGC repeat expansion in NOTCH2NLC. The repeat expansion length was estimated using CRISPR/Cas9-based targeted long-read sequencing. The three patients (two PD patients from Family 1 and one sporadic PD) carrying the pathogenic NOTCH2NLC expansion were reconfirmed with a diagnosis of clinically established PD. Although they lacked the typical neuronal intranuclear inclusion disease (NIID) magnetic resonance imaging (MRI) feature, the typical PD pattern of striatal dopamine transporter loss was detected. Notably, all three patients presented with systemic areflexia, and other secondary causes of polyneuropathy were excluded. Skin biopsy showed intranuclear inclusions and an absence of phosphorylated alpha-synuclein deposition in the skin nerve fibers of all three patients. Although these clinically diagnosed PD patients with pathogenic GGC repeat expansion in NOTCH2NLC were hardly distinguishable from idiopathic PD based on clinical course and neuroimaging features, the pathological findings indicated that their phenotype was a PD phenocopy of NIID. Systemic areflexia may be an important and unique clinical clue suggesting further genetic testing and skin biopsy examination to confirm the diagnosis of NIID in patients presenting with a PD phenocopy.

Experimental and modeling investigation of critical slugging behavior in marine compressed gas energy storage systems

As a flexible-scale energy storage technology, underwater compressed gas energy storage is an emerging enabler of the transition to renewable and sustainable energy structure. Liquid accumulation often occurs in the process of underwater pipeline gas transmission, posing a challenge that must be overcome. In this study, an experimental investigation is presented to investigate the flow characteristics of liquid in a hilly-terrain tube with zero net liquid flow. By adjusting the gas velocity, pipe inclination, liquid accumulation volume, and measuring the expansion length of the liquid film in the horizontal pipe, the liquid accumulation movement is divided into three development stages, which are related to the emergence of the liquid slug. The liquid slug in the pipe is moved by absorption, consolidation, and outflow. The movement of the liquid slug is captured by a high-speed camera, and the formation mechanism of the liquid slug is divided into three categories. When the gas flow rate is low, a liquid slug is formed due to wave growth and convective extrusion. The liquid slug produced by wave merging occurs when the gas velocity is high. A theoretical model of the slugging velocity is established. The relationship between maximum growth factor, liquid film thickness, and gas velocity is analyzed. Furthermore, the critical slugging velocity is determined. The model predictions agreed with the experimental measurement results, with a maximum error of less than 10%. This work can help in furthering underwater compressed air energy storage technology.

A Study on the Effect of Fiber Volume Ratio on the Physical and Mechanical Properties of Natural Fiber Composites

Abstract: At present, natural fibers of renewable resources offer the potentiality to act like a reinforcing material for manufacturing of composites. This study provides the effect of fiber volume ratio on physical & mechanical properties of specimen when the fiber length is fixed and randomly oriented. For the fabrication of composite samples, the natural fiber was used as a reinforced material and adhesive material was Phenol-Formaldehyde. Fibers of hemp, coconut & jute are used in equal ratio for the research work. In this study the fiber length was fixed in all samples and the fibers were randomly orientated and the thickness of all samples was 5mm. The length of the fibers was 12mm. A total of four samples S1, S2, S3, and S4 were fabricated using hand lay-up method. Fiber volume ratio is varied from 30% to 60%, such as 30% in S1, 40%in S2, 50% in S3 & 60% in S4 sample. Physical & Mechanical Testing has been carried out as per standards on the prepared specimens. Water absorption test was carried out in two sets one for 2 hours & second for 24 hours. Result shows that fiber volume ratio of 40% in S2 showed maximum density, water absorption test was found minimum for 2 and 24 hours test & minimum moisture. At 30% fiber ratio (S1) the expansion in length, width, and thickness was found minimum during the dimension expansion test for 2 hour soaking. Modulus of elasticity of the composite was found maximum at 50% fiber ratio (S3) inside composite and modulus of rupture found maximum at 60% fiber ratio (S4). The study on the effect of fiber ratio on mechanical and physical properties has been done.

Asymmetric Twisting of C-Centered Octahedral Gold(I) Clusters by Chiral N-Heterocyclic Carbene Ligation.

Asymmetric induction of metal clusters by ligation of chiral ligands is intriguing in terms of the mechanism of chirality transfer and the stability of the resulting chiral structure. Here we report the asymmetric induction of C-centered hexagold(I) CAuI6 clusters into an asymmetrically twisted structure through monodentate, chiral benzimidazolylidene-based N-heterocyclic carbene (NHC) ligands. X-ray diffraction analysis revealed that the NHC-ligated CAuI6 cluster was diastereoselectively twisted with directionally selective, bond length expansion, and contraction of the Au···Au contacts and that the original cluster with high symmetry was transformed into an optically pure, asymmetric CAuI6 cluster with C1 symmetry. Moreover, the circular dichroism spectroscopy and the time-dependent density functional theory calculation confirmed that the asymmetrically twisted CAuI6 structure was maintained even in solution. Such asymmetric induction of configurationally stable metal clusters would greatly expand the molecular design possibilities of asymmetric catalysts and chiroptical materials by utilizing library chiral NHC ligands.

Development of length expansion temperature detector based on arduino uno

Length expansion is an increase in the length of an object due to receiving heat. If a solid object is heated then the object will expand in all directions, in other words the length increases the length of an object because it receives heat. A tool for comparing the length expansion of various metals is the Musschenbrock. To measure the temperature of an object can use a sensor. The sensor used in this research is the LM 35 based on Arduino Uno. The development method for this Length expansion tool temperature detector starts from tool design, hardware design, software design, tool calibration and tool testing. From the results of research using an Arduino Uno-based temperature detector, it can be said that this research was successful because the values obtained were almost the same as the existing theory. So, this detector is suitable for use in laboratories or schools as a learning medium to determine the length expansion of metals.

On the SEL Egyptian fraction expansion for real numbers

<abstract><p>In the authors' earlier work, the SEL Egyptian fraction expansion for any real number was constructed and characterizations of rational numbers by using such expansion were established. These results yield a generalized version of the results for the Fibonacci-Sylvester and the Engel series expansions. Under a certain condition, one of such characterizations also states that the SEL Egyptian fraction expansion is finite if and only if it represents a rational number. In this paper, we obtain an upper bound for the length of the SEL Egyptian fraction expansion for rational numbers, and the exact length of this expansion for a certain class of rational numbers is verified. Using such expansion, not only is a large class of transcendental numbers constructed, but also an explicit bijection between the set of positive real numbers and the set of sequences of nonnegative integers is established.</p></abstract>

Association of nutritional, cooking quality and yield traits in F1 and F2 population of rice (Oryza sativa L.)

The magnitude of association between nutritional, cooking quality, yield and its attributing characters in nutrient rich rice genotype crosses studied during Kharif and Rabi seasons grain yield per plant exhibited significant correlations between the number of grains per plant, test weight, kernel length and L/B ratio in both F1 and F2 generations, respectively. The association analysis with cooking and nutritional quality traits indicated existence of a significant positive association between kernel length and volume expansion ratio and also with kernel elongation ratio. The study further confirmed that there is significant correlation between gelatinization temperature and cooking quality traits besides amylase and protein contents. Zinc and iron contents were negatively correlated with cooking quality traits viz., kernel length, volume expansion ratio, kernel elongation ratio and with amylase content. The correlation between zinc and iron content is significantly positive.

Effect of limestone fines as a partial replacement of cement on the chemical, autogenous, drying shrinkage and expansion of mortars

The use of limestone with its different applications in paste, mortar, and concrete has become a common practice, not only for the urge to control the environmental impact and conserving the virgin materials but also to improve their performance in different environment. This study focused on the effect of incorporating different percentage levels of limestone fines (LF) on the shrinkage and expansion development of mortar specimens for a total period of 28 days. To observe this effect, a total of 5 mortar mixes were employed with five substitution rate of cement with LF ranging from 0 to 20%. The ratios of water to binder and sand to binder remain constant at 0.45 and 2 respectively. Testing consisted of three types of length change; chemical, drying and autogenous shrinkage as well as expansion. It was observed that adding up to 10% LF enhanced the chemical and autogenous shrinkage of mortars. Likewise, incorporating up to 10% LF increased the expansion in mortar specimens. However, drying shrinkage of mortars increased as LF content went up. Overall, the results indicate that there is strong correlation between the chemical shrinkage and the other length change parameters and expansion.

Properties of blade-like field emitters

Blade-Like Field Emitters (BFE), as defined here, are emitters expanded in one direction, forming a sharp emitting edge instead of a sharp tip. These structures have four main advantages compared to their needle counterparts, i.e., they are mechanically firmer, are better electrical and thermal conductors, and provide a larger emission area. We focus on the optimization of the last of these. We evaluate the emission properties of three types of BFEs, which we short-named hSoC-blade, HCP-blade and Elli-blade. Each is built from the expansion of a hemisphere-on-a-cone (hSoC), hemisphere-on-a-cylindrical-post (HCP) and an ellipsoidal (Elli) emitter, respectively. The characteristics of the field enhancement factor, the local electrostatic field distribution on each blades’ edges and their notional area (An) of emission as a function of the expansion length are described. Finally, we point out how to improve the edge of the HCP-blade to obtain the optimal profile, which yield the largest An.

Longer CAG repeat length is associated with shorter survival after disease onset in Huntington disease

It is well known that the length of the CAG trinucleotide expansion of the huntingtin gene is associated with many aspects of Huntington disease progression. These include age of clinical onset and rate of initial progression of disease severity. The relationship between CAG length and survival in Huntington disease is less studied. To address this, we obtained the complete Registry HD database from the European Huntington Disease Network and reanalyzed the time from reported age of disease onset until death. We conducted semiparametric proportional hazards modeling of 8,422 participants who had experienced onset of clinical Huntington disease, either retrospectively or prospectively. Of these, 826 had a recorded age of death. To avoid biased model estimates, retrospective onset ages were represented by left truncation at study entry. After controlling for onset age, which tends to be younger in those with longer CAG repeat lengths, we found that CAG length had a substantial and highly significant influence upon survival time after disease onset. For a fixed age of onset, longer CAG expansions were predictive of shorter survival. This is consistent with other known relationships between CAG length and disease severity. We also show that older onset age predicts shorter lifespan after controlling for CAG length and that the influence of CAG on survival length is substantially greater in women. We demonstrate that apparent contradictions between these and previous analyses of the same data are primarily due to the question of whether to control for clinical onset age in the analysis of time until death.

Evaluation and Load Rating of a Steel Girder In-Span Hinge Connection

Continuous steel plate girder bridges often use intermediate expansion joints located at in-span hinges to divide the superstructure into individual units with shorter expansion lengths. One common type of in-span hinge is often termed a “shiplap joint.” This type of joint is located away from piers near the moment inflection point of the span, maximizing girder efficiency. It consists of a cantilevered portion of the superstructure supporting a suspended portion of the latter on bearings placed on dapped portions of the steel plate girders. Few references are available for the evaluation and load rating of shiplap in-span hinges used in steel girder bridges. In this study, the strength and stability of a typical shiplap hinge connection is evaluated using two methodologies: a 3D finite element model including a detailed mesh of the connection; and a proposed simplified methodology based on design equations. Load ratings of the connections based on these methodologies are compared. The proposed approach allows for a conservative assessment of the hinge without the need for a detailed finite element model.

Comparison of TCF4 repeat expansion length in corneal endothelium and leukocytes of patients with Fuchs endothelial corneal dystrophy.

Expansion of CTG trinucleotide repeats (TNR) in the transcription factor 4 (TCF4) gene is highly associated with Fuchs Endothelial Corneal Dystrophy (FECD). Due to limitations in the availability of DNA from diseased corneal endothelium, sizing of CTG repeats in FECD patients has typically been determined using DNA samples isolated from peripheral blood leukocytes. However, it is non-feasible to extract enough DNA from surgically isolated FECD corneal endothelial tissue to determine repeat length based on current technology. To circumvent this issue, total RNA was isolated from FECD corneal endothelium and sequenced using long-read sequencing. Southern blotting of DNA samples isolated from primary cultures of corneal endothelium from these same affected individuals was also assessed. Both long read sequencing and Southern blot analysis showed significantly longer CTG TNR expansion (>1000 repeats) in the corneal endothelium from FECD patients than those characterized in leukocytes from the same individuals (<90 repeats). Our findings suggest that the TCF4 CTG repeat expansions in the FECD corneal endothelium are much longer than those found in leukocytes.

Coupled effect of expansive agent and curing on mechanical and shrinkage properties of fiber-reinforced Eco-Crete

This study evaluates the effectiveness of various shrinkage mitigating strategies on the properties of economical and ecological concrete (referred to here as Eco-Crete) made with a low binder content and 55% substitution of cement with fly ash and slag cement. Shrinkage mitigating strategies were included the use of Type G expansive agent (EA), saturated lightweight sand (LWS) for internal curing, and 0.5% micro–macro steel fibers. The coupled effect of these materials along with external moist curing (MC) of 1 and 14 days on mechanical and shrinkage properties was investigated. Eight plain and eight fiber-reinforced Eco-Crete mixtures were tested for compressive strength, flexural strength, drying shrinkage, and restrained expansion. Induced stress resulting from the restrained expansion of the Eco-Crete mixtures was determined, and a multiple regression model was developed to estimate the internally induced stress. MC, EA content, fiber volume, and coupled effect of EA-fiber were found to be significant in enhancing the induced stress. The Eco-Crete mixtures including 10% EA and 14 days of MC exhibited the maximum initial expansion and minimum shrinkage in both shrinkage and restrained expansion length change experiments. The coupled effect of EA and fiber reduced 112-d shrinkage and restrained expansion length change by up to 26% and 54%, respectively, compared to the system made with EA, LWS, and fiber. The highest induced stress and flexural strength of 25 kPa and 6.2 MPa, respectively, were obtained by the mixture made with 10% EA and 0.5% fiber.

The complete mitochondrial genome of Ophiocordyceps gracilis and its comparison with related species

In this study, the complete mitochondrial genome of O. gracilis was sequenced and assembled before being compared with related species. As the second largest mitogenome reported in the family Ophiocordycipitaceae, the mitogenome of O. gracilis (voucher OG201301) is a circular DNA molecule of 134,288 bp that contains numerous introns and longer intergenomic regions. UCA was detected as anticodon in tRNA-Sec of O. gracilis, while comparative mitogenome analysis of nine Ophiocordycipitaceae fungi indicated that the order and contents of PCGs and rRNA genes were considerably conserved and could descend from a common ancestor in Ophiocordycipitaceae. In addition, the expansion of mitochondrial organization, introns, gene length, and order of O. gracilis were determined to be similar to those of O. sinensis, which indicated common mechanisms underlying adaptive evolution in O. gracilis and O. sinensis. Based on the mitochondrial gene dataset (15 PCGs and 2 RNA genes), a close genetic relationship between O. gracilis and O. sinensis was revealed through phylogenetic analysis. This study is the first to investigate the molecular evolution, phylogenetic pattern, and genetic structure characteristics of mitogenome in O. gracilis. Based on the obtained results, the mitogenome of O. gracilis can increase understanding of the genetic diversity and evolution of cordycipitoid fungi.

Alternative method for computing primary wavefront aberrations using Taylor series expansion of optical path length

This work proposes a methodology for computing the primary wavefront aberration coefficients of axis-symmetrical optical systems by expanding the optical path length of a general ray using the Taylor series expansion. The obtained values of the wavefront aberration coefficients are compared with those determined from Zemax simulations. It is shown that the corresponding numerical results are in excellent agreement with those obtained from Zemax simulations.

Neuroinflammation in the Cerebellum and Brainstem in Friedreich Ataxia: An [18F]-FEMPA PET Study.

Neuroinflammation is proposed to accompany, or even contribute to, neuropathology in Friedreich ataxia (FRDA), with implications for disease treatment and tracking. To examine brain glial activation and systemic immune dysfunction in people with FRDA and quantify their relationship with symptom severity, duration, and onset age. Fifteen individuals with FRDA and 13 healthy controls underwent brain positron emission tomography using the translocator protein (TSPO) radioligand [18 F]-FEMPA, a marker of glial activation, together with the quantification of blood plasma inflammatory cytokines. [18 F]-FEMPA binding was significantly increased in the dentate nuclei (d=0.67), superior cerebellar peduncles (d=0.74), and midbrain (d=0.87), alongside increased plasma interleukin-6 (IL-6) (d=0.73), in individuals with FRDA compared to controls. Increased [18 F]-FEMPA binding in the dentate nuclei, brainstem, and cerebellar anterior lobe correlated with earlier age of symptom onset (controlling for the genetic triplet repeat expansion length; all r part < -0.6), and in the pons and anterior lobe with shorter disease duration (r=-0.66; -0.73). Neuroinflammation is evident in brain regions implicated in FRDA neuropathology. Increased neuroimmune activity may be related to earlier disease onset and attenuate over the course of the illness. © 2021 International Parkinson and Movement Disorder Society.

Nystagmus may be the first neurological sign in early stages of spinocerebellar ataxia type 3.

Spinocerebellar ataxia type 3 (SCA3) is the most common autosomal dominant spinocerebellar ataxia worldwide. Almost all patients with SCA3 exhibit nystagmus and/or saccades impairment. To investigate the presence of nystagmus as an early neurological manifestation, before ataxia, in some patients with SCA3 in the first six months of the disease. We evaluated a series of 155 patients with clinically and molecularly proven SCA3 between 2013 and 2020. Data regarding sex, age, age at onset, disease duration, CAG repeat expansion length, first symptom, presence of ataxia, scores on SARA and ICARS scales, and presence and characteristics of nystagmus were collected. We identified seven patients with symptomatic SCA3 who presented with isolated nystagmus. In these seven individuals the age at onset ranged from 24 to 57 years, and disease duration from four to six months. Our study showed that nystagmus may be the first neurological sign in SCA3. This clinical observation reinforces the idea that the neurodegenerative process in SCA3 patients may start in vestibular system connections or in flocculonodular lobe. This study adds relevant information about pre-symptomatic features in SCA3 that may work as basis for a better understanding of brain degeneration and for future therapeutic clinical trials.

Investigation on the Maximum Expansion Length of Urban Rail Transit Double-Tower Cable-Stayed Bridges Without Rail Expansion Device

Cable-stayed bridges carrying urban rail transit are often equipped with rail expansion devices (REDs) to avoid possible breaking or buckling of the rails. Such devices not only increase construction and maintenance budgets of the track system, but also endanger ride comfort and safety of the trains. Therefore, the maximum expansion length of cable-stayed bridges without the adoption of RED is investigated and presented in this paper. Finite element models of cable-stayed bridges with various lengths are developed based on design parameters of existing bridges with similar lengths. A friction-spring model is utilized to simulate the nonlinear resistance history of fasteners under various loading cases. Track–bridge interactions are then studied by varying the longitudinal restraint conditions for the cable-stayed bridges. The investigation reveals that the admissible overall rail stress allows a maximum expansion length of about 400[Formula: see text]m for urban rail transit double-tower cable-stayed bridge paved with the ballastless track and WJ-2 small resistance fasteners. This finding provides theoretical bases and practical guidelines for the saving of REDs for many large-span urban rail transit bridges in future design.

Compression-controlled dynamic buckling in thin soft sheets.

We investigate experimentally the dynamic phase transition from compressed to buckled phases for thin sheets of rubber. We find that the rubber strips enter a highly compressed, metastable state when compression speed is high. During the compressed phase, higher modes grow, followed by mode coarsening. Mode growth is accompanied by an expansion of length while the system is still being compressed. We measure the forces and length of the sheet to confirm this, and we develop a mechanism for how modes grow and coarsen during dynamical buckling. The influence of crucial control parameters in the experiments, such as the material cross section and compression speed, on the buckling dynamics, are explained theoretically.

Kinematics of femtosecond laser-generated plasma expansion: Determination of sub-micron density gradient and collisionality evolution of over-critical laser plasmas

An optical diagnostic based on resonant absorption of laser light in a plasma is introduced and is used for the determination of density scale lengths in the range of 10 nm to &amp;gt;1 μm at the critical surface of an overdense plasma. This diagnostic is also used to extract the plasma collisional frequency, allowing inference of the temporally evolving plasma composition on the tens of femtosecond timescale. This is found to be characterized by two eras: the early time and short scale length expansion (L &amp;lt; 0.1λ), where the interaction is highly collisional and target material dependent, followed by a period of material independent plasma expansion for longer scale lengths (L &amp;gt; 0.1λ); this is consistent with a hydrogen plasma decoupling from the bulk target material. Density gradients and plasma parameters on this scale are of importance to plasma mirror optical performance and comment is made on this theme.