Deciphering the modifiers for phenotypic variability of X-linked adrenoleukodystrophy

X-linked adrenoleukodystrophy(X-ALD),an inborn error of peroxisomalβ-oxidation,is caused by defects in the ATP Binding Cassette Subfamily D Member 1(ABCD1)gene.X-ALD patients may be asymptomatic or present with several clinical phenotypes varying from severe to mild,severe cerebral adrenoleukodystrophy to mild adrenomyeloneuropathy(AMN).Although most female heterozygotes present with AMN-like symptoms after 60 years of age,occasional cases of females with the cerebral form have been reported.Phenotypic variability has been described within the same kindreds and even among monozygotic twins.There is no association between the nature of ABCD1 mutation and the clinical phenotypes,and the molecular basis of phenotypic variability in X-ALD is yet to be resolved.Various genetic,epigenetic,and environmental influences are speculated to modify the disease onset and severity.In this review,we summarize the observations made in various studies investigating the potential modifying factors regulating the clinical manifestation of X-ALD,which could help understand the pathogenesis of the disease and develop suitable therapeutic strategies.

Effects of Plasticizers,Antioxidants and Burning Rate Modifiers on Aging Performance of the HTPB/HMDI Composite Solid Propellant

The effects of plasticizers,antioxidants and burning rate modifiers on the aging performance of the composite solid propellant based on hydroxyl-terminated polybutadiene(HTPB)/hexamethylene diisocyanate(HMDI)were explored by apply-ing an accelerated aging program for 90 day at 70 ℃. The HTPB propellant matrix with the diisooctyl sebacate(DOS)as plasti-cizers and diisooctyl azelate(DOZ), antioxidants as N,N ′-Diphenyl-p-phenylenediamine(AO) and 2,2′-methylenebis(4-methyl-6-tert-butylphenol)(cyanox 2246)and burning rate modifiers as barium ferrite(BF),copper chromites(CC)and fer-ric oxide(FO)were varied. Results show that sample(S1)which based on DOS decreases the stress value and increases the strain value which considered to be an excellent start for aging program. Sample(S3)containing AO presents the higher resis-tance to oxidation showing the better performance that reflects on increasing the shelf life of the composite solid propellant mo-tor. Sample(S5)which based on BF enhances the ballistic performance among over the other tested two samples. The accelerat-ed aging program allowed us to estimate the motor in-service lifetime.

Influence of Modifiers on Supercritical Fluid Chromatography (SFC) and Supercritical Fluid Extraction (SFE), Part I

It is important to understand the mechanism and implications of different modifiers on analytical and preparative processes under chromatography with supercritical fluids (SFs) and under extraction with SFs. Supercritical fluid chromatography (SFC) and supercritical fluid extraction are generally carried out with neat supercritical carbon dioxide (SCCO2) or with SCCO2 containing modifiers (or cosolvents), especially for strongly polar compounds. For example, methanol is added as a cosolvent/modifier to SCCO2 for the extraction/separation of polar compounds. This paper discusses the influence of the modifier on the colligative properties of the principal mobile phase, which may define the situation in the total mobile phase in a chromatography column or in parts of a column under SFC. No colligative behavior of solutions reflects individual properties of the solutes. Their cross-interactions with solvents are discussed.

Influence of Modifiers, Extractants, and Trappers on Lipid Composition with Liquids in Standard State Extraction, Supercritical Fluid Extraction and Trapping by Supercritical Fluid Extraction, Part II

Modifiers have a broad array of influences on extraction with liquids in standard state, supercritical fluid extraction (SFE), trapping by SFE and supercritical fluid chromatography (SFC). They can significantly change the qualitative and quantitative results. Quantitative and qualitative results can be influenced by different extractants and modifiers in different ways as it was shown by Brondz et al. at 2007 in “The real nature of the indole alkaloids in Cortinarius infractus: Evaluation of artifact formation through solvent extraction method development”, J. Chromatography A, 1148, 1-7. The choice of correct extractant, modifier, and trapper to the bulk mobile phase for supercritical fluids (SFs) or for liquids in subcritical or in the liquids in standard state is a challenge in any extraction procedure. This is the second paper in a sequence that describes the influence of extractants and modifiers on the performance of SFs and results of extraction with liquids in standard state and SFE. Here, attention is given to possible mistakes in qualitative and quantitative results by poor understanding of the influence of extractants, modifiers, and trappers on extraction and trapping process by a careless choice of extractant, modifier, and trapper for extraction with liquids in standard state and SFE. The SF chosen for discussion in the paper is CO2. However, similar effects can be observed with use of other SFs and fluids in subcritical and standard states. In this paper, the discussion of lipids, fatty and carboxylic acids have been chosen as target analytes for extraction, trapping and analysis. Some examples from extraction with liquids in the standard state and trapping in the supercritical state (collection) have been furnished with the wrong extractant, modifier, or trapper which is presented for illustration of inappropriate choice of extractants, modifiers, and trappers.

INFLUENCES OF MODIFIERS ON SURFACE PROPERTIES OF WHITE CARBON BLACK AND MECHANICAL PROPERTIES OF SILICONE RUBBER

Various of modifiers were used to modify the surface activity of white carbon black. The oil absorption, viscosity, hydrophobic rate and burning loss of white carbon black and the mechanical propertiess of silicone rubber were measured. The influences of the modifiers on the properties of white carbon black and the mechanical properties of silicone rubber were discussed.

Morphology Control of SrCO_3 Crystals using Complexons as Modifiers in the Ethanol-water mixtures

Using SrC12-6H2O and Na2CO3 as the main raw materials and adding different complexons as modifiers with simple co-precipitation method, SrCO3 crystals with distinct morphologies like spherical, bundle-like, overlapping plate-like, hexagonal star-like, dumbbell-like, etc. can be synthesized in the ethanol-water mixtures. The samples were characterized by X-ray diffraction （XRD）, scanning electron microscopy （SEM） and Fourier transform infrared spectrograph （FT-IR）. The interrelated effect mechanism is presented in the end. Results show that the modifier carboxyl groups play a significant role in controlling the SrCO3 crystal morphologies, which can alter the crystal growth unit （Sr^2＋） supply mode and induce the crystal formation with the morphologies matching their spatial configurations.

Effects of Modifiers on the Anti-wetting and Anti-icing Property of Aluminum Surface

The effects of modifiers on the anti-wetting and anti-icing property of the prepared rough aluminum surface were investigated.The rough aluminum substrates were obtained through electrochemical oxidization with 15 wt% sulfuric acid solution as the electrolyte at the constant current of 4 mA for 3 h.And then they were modified with octadecanoic acid (C18),polyethylene (PE),polystyrene (PS),polyethylene glycol (PEG) and hexamethylenetetramine (HMTA),respectively,whose surface free energies were 27.6,31.0,33.0,61.6 and 70.0 mN/m,respectively.The contact angles (CA) were 154.6°,128.4°,127.6°,5.0° and 0.0°,respectively,and the ice adhesion pressures were 15.9,36.3,55.9,155.3 and 216.1 kPa,respectively.The ice adhesion strengths decrease along with the increasing anti-wetting property of aluminum surfaces and the decreasing of the surface energy of modifiers.These provide some new insights when designing the aluminum surface with anti-icing properties in some special applications.

Influence of SBS modifiers on viscoelastic mechanical behavior of modified asphalt

In order to verify the influence of different block proportions S/B on the effect of SBS modified asphalt,the dynamic mechanical performance test and static loading test were performed on the samples composed of different kinds of SBS with base asphalt. It is found that different S/B values fix on different modified effects and different viscoelastic mechanical behaviors,due to biphasic separate fabric of polybutadiene and polystyrene in SBS. In low-speed running pavement,the modified asphalt with lower S/B value shows better pavement performance,while in high-speed running pavement,the modified asphalt with higher S/B value shows better pavement performance. As far as SBS modified asphalt itself is concerned,mixing proportion impacts on resisting displacement and block proportion S/B ratio impacts on strain recovery capacity. In the case that the conditions are the same,SBS modified asphalt with different S/B values can be used for different travelling speed pavement construction demands to get an intelligent use.

Measurements of the Electrical Incidence Angle Modifiers of an Asymmetrical Photovoltaic/Thermal Compound Parabolic Concentrating-Collector

Reflector edges, sharp acceptance angles and by-pass diodes introduce large variations in the electrical performance of asymmetrical concentrating photovoltaic/thermal modules over a short incidence angle interval. It is therefore important to quantify these impacts precisely. The impact on the electrical performance of the optical properties of an asymmetrical photovoltaic/thermal CPC-collector was measured in Maputo, Mozambique. The measurements were carried out with the focus on attaining a high resolution incidence angle modifier in both the longitudinal and transversal directions, since large variations were expected over small angle intervals. A detailed analysis of the contribution of the diffuse radiation to the total output was also carried out. The solar cells have an electrical efficiency of 18% while the maximum measured electrical efficiency of the collector was 13.9 % per active glazed area and 20.9 % per active cell area, at 25 °C. Such data make it possible to quantify not only the electrical performance for different climatic and operating conditions but also to determine potential improvements to the collector design. The electrical output can be increased by a number of different measures, e.g. removing the outermost cells, turning the edge cells 90°, dividing each receiver side into three or four parts and directing the tracking, when used, along a north-south axis.

Effect of Different Organic Modifiers on the Tensile Properties of PVC/EVA/Montmorillonite Composites

Poly (vinyl chloride)/ethylene-vinyl acetate/montmorillonite (PVC/EVA/OMMT) composites were prepared by melt blending method. Two kinds of montmorillonites were organically modified by trimethyloctadecyl ammonium and dimethyl bis (hydrogenated tallow) ammonium, respectively. The morphology and tensile properties of the resultant composites were discussed in terms of the modifier type and OMMT content. The PVC/EVA/OMMT composites have intercalated structure, which is independent of the polarity of the modifiers, while the tensile properties show strong dependence on the modifier type. The OMMT modified by polar modifier gives higher tensile ductile and strength of PVC/EVA/OMMT composites.

Effect of Network Modifiers on Spectroscopic Properties of Erbium-doped Phosphate Glasses

The integrated absorption cross section Σ abs, peak emis sion cross section σ emi, Judd-Ofeld intensity parameters Ω t(t=2,4,6), and spontaneous emission probability A R of Er 3+ ions were determined fo r Erbium doped alkali and alkaline earth phosphate glasses. It is found the comp ositional dependence of σ emi is almost similar to that of Σ abs, wh ich is determined by the sum of Ω t (3Ω 2+10Ω 4+21Ω 6). In addition, the compositional dependence of Ω t was studied in these glass systems. As a resu lt, compared with Ω 4 and Ω 6, the Ω 2 has a stronger compositional depend ence on the ionic radius and content of modifiers. The covalency of Er-O bonds in phosphate glass is weaker than that in silicate glass, germanate glass, alumi nate glass, and tellurate glass, since Ω 6 of phosphate glass is relatively la rge. A R is affected by the covalency of the Er 3+ ion sites and correspon ds to the Ω 6 value.

Application of CX-type modifiers in Al-Si alloys

The modification effect of CX-type (CX means the modifiers that have longeffective term) modifiers applied in ZL108 and ZL104 Al-Si alloys has been studied in detail. Theresults show that the morphologies of the eutectic silicon and the primary silicon can be modifiedand refined simultaneously. The modification effect acts quickly and can maintain a period of 8 h bythe CX-type modifiers. The CX-type modifiers increase the mechanical properties of Al-Si alloys andimprove the service properties of machine parts made of Al-Si alloys (such as piston and cylinderetc.). In modifying processes, the amount of the addition of CX-type modifiers is smaller than thatof any other modifiers, and the modifying procedures are simple. There are no smoke, no dust, and noirritant smell in modifying processes using CX-type modifiers. Therefore, the CX-type modifiershave advantages in economy and environment protection.

An experimental study on the effects of friction modifiers on wheel-rail dynamic interactions with various angles of attack

By modifying friction to the desired level,the application of friction modifiers(FMs)has been considered as a promising emerging tool in the railway engineering for increasing braking/traction force in poor adhesion conditions and mitigating wheel/rail interface deterioration,energy consumption,vibration and noise.Understanding the effectiveness of FMs in wheel–rail dynamic interactions is crucial to their proper applications in practice,which has,however,not been well explained.This study experimentally investigates the effects of two types of top-of-rail FM,i.e.FM-A and FM-B,and their application dosages on wheel–rail dynamic interactions with a range of angles of attack(AoAs)using an innovative well-controlled V-track test rig.The tested FMs have been used to provide intermediate friction for wear and noise reduction.The effectiveness of the FMs is assessed in terms of the wheel–rail adhesion characteristics and friction rolling induced axle box acceleration(ABA).This study provides the following new insights into the study of FM:the applications of the tested FMs can both reduce the wheel–rail adhesion level and change the negative friction characteristic to positive;stick–slip can be generated in the V-Track and eliminated by FM-A but intensified by FM-B,depending on the dosage of the FMs applied;the negative friction characteristic is not a must for stick–slip;the increase in ABA with AoA is insignificant until stick–slip occurs and the ABA can thus be influenced by the applications of FM.

Tendency of Modifiers in English Noun Phrases from the Perspective of Cognition

There are both premodifiers and postmodifiers English noun phrases.Premodifiers and postmodifiers can be change able along with the adjustments of human cognition.It discusses the cognitive foundation of transformation between postmodifi ers and premodifiers in English noun phrases so as to find out the cognitive reason behind linguistic structure.

Molecules with a TEMPO-based head group as high-performance organic friction modifiers

High-performance organic friction modifiers(OFMs)added to lubricating oils are crucial for reducing energy loss and carbon footprint.To establish a new class of OFMs,we measured the friction and wear properties of N-(2,2,6,6-tetramethyl-1-oxyl-4-piperidinyl)dodecaneamide referred to as C12Amide-TEMPO.The effect of its head group chemistry,which is characterized by a rigid six-membered ring sandwiched by an amide group and a terminal free oxygen radical,was also investigated with both experiments and quantum mechanical(QM)calculations.The measurement results show that C12Amide-TEMPO outperforms the conventional OFMs of glyceryl monooleate(GMO)and stearic acid,particularly for load-carrying capacity,wear reduction,and stability of friction over time.The friction and wear reduction effect of C12Amide-TEMPO is also greatly superior to those of C12Ester-TEMPO and C12Amino-TEMPO,in which ester and amino groups replace the amide group,highlighting the critical role of the amide group.The QM calculation results suggest that,in contrast to C12Ester-TEMPO,C12Amino-TEMPO,and the conventional OFMs of GMO and stearic acid,C12Amide-TEMPO can form effective boundary films on iron oxide surfaces with a unique double-layer structure:a strong surface adsorption layer owing to the chemical interactions of the amide oxygen and free radical with iron oxide surfaces,and an upper layer owing to the interlayer hydrogen-bonding between the amide hydrogen and free radical or between the amide hydrogen and oxygen.Moreover,the intralayer hydrogen-bonding in each of the two layers is also possible.We suggest that in addition to strong surface adsorption,the interlayer and intralayer hydrogen-bonding also increases the strength of the boundary films by enhancing the cohesion strength,thereby resulting in the high tribological performance of C12Amide-TEMPO.The findings in this study are expected to provide new hints for the optimal molecular design of OFMs.

Density functional theory for selecting modifiers for enhanced adsorption of tetracycline in water by biochar

Antibiotics and their metabolic byproducts are found in wastewater and natural water as a result of increased consumption,posing a major threat to humans and other living organisms.One of the most promising methods for their removal is adsorption using biochar because it offers excellent adsorption potential and is both affordable and environmentally beneficial.However,raw biochar frequently has a low adsorption capacity due to its limited pore structure and unfavorable surface characteristics.Biochar surface modifications using modifiers such as H3PO4,KOH,and NaOH have improved the surface area and thereby the adsorption capacity.Experimental methods for assessing the effectiveness and adsorption mechanism of modified biochar are costly and time-consuming.Density functional theory(DFT)was used to investigate the interfacial interactions and adsorption mechanism of tetracycline(TC),a widely used antibiotic for personal care and veterinary medication,on unmodified and modified biochar.The DFT calculations showed that the adsorption energy of TC on unmodified and modified biochar is in the following order:KOH-modified biochar(−2.38 eV)<NaOH-modified biochar(−2.20 eV)<unmodified biochar(−1.56 eV)<H3PO4-modified biochar(5.48 eV).The lower adsorption energy is associated with a stronger and more stable interaction between the adsorbent and the contaminant.This suggests that the adsorption of TC on KOH-modified biochar is more prolific and stable compared to the other biochar.This study provides an understanding of the mechanism underlying the adsorption of TC by modified biochar and can be used as a guide to screen for biochar with promising adsorption potential prior to experimental efforts.

A dual-RPA based lateral flow strip for sensitive,on-site detection of CP4-EPSPS and Cry1Ab/Ac genes in genetically modified crops

Traditional transgenic detection methods require high test conditions and struggle to be both sensitive and efficient.In this study,a one-tube dual recombinase polymerase amplification(RPA)reaction system for CP4-EPSPS and Cry1Ab/Ac was proposed and combined with a lateral flow immunochromatographic assay,named“Dual-RPA-LFD”,to visualize the dual detection of genetically modified(GM)crops.In which,the herbicide tolerance gene CP4-EPSPS and the insect resistance gene Cry1Ab/Ac were selected as targets taking into account the current status of the most widespread application of insect resistance and herbicide tolerance traits and their stacked traits.Gradient diluted plasmids,transgenic standards,and actual samples were used as templates to conduct sensitivity,specificity,and practicality assays,respectively.The constructed method achieved the visual detection of plasmid at levels as low as 100 copies,demonstrating its high sensitivity.In addition,good applicability to transgenic samples was observed,with no cross-interference between two test lines and no influence from other genes.In conclusion,this strategy achieved the expected purpose of simultaneous detection of the two popular targets in GM crops within 20 min at 37°C in a rapid,equipmentfree field manner,providing a new alternative for rapid screening for transgenic assays in the field.

Effect of organic modifiers on the capacity factor in micellar electrokinetic chromatography

Organic modifiers were effective both to extend the migration time window and to improve the separation of very hydrophobic compounds in MEKC. An iteration method was used to determine the migration time of micelles. The quantitative relationship between the capacity factor k' and the concentration of organic modifiers was derived, which was investigated experimentally. The linear solvation energy relationships (LSER) methodology was applied to MEKC, and good linear relationships between lnk' and solvatochromic parameters of 15 solutes were obtained in the presence of organic modifier in different concentrations, which indicated a new access in MEKC to predict k' from the structural parameters of solutes. The effect of column temperature T on k' was also investigated.

Utilization of recycled solid waste as ecological reclamation materials based on plant growth experiments and soil quality analysis

During the highway construction,a large number of spoil areas will be generated while a large amount of waste slag and soil could not be rationally utilized.Besides,the vegetation recovery is slow in the spoil areas due to the soil depletion.Aiming at recycling the solid waste,the sieved engineering waste slag with local red clay and corn straw biochar was supplied to solve the problem of insufficient nutrients in engineering waste slag and soil.In addition,planting experiments of alfalfa(Medicago sativa L.)and Amorpha fruticosa L.combined with physical and chemical experiments were carried out to prove the feasibility of the novel improved substrate for the reclamation of spoil areas.The results show that the substrate's improvement effect is mainly affected by the soil to slag ratio and the biochar content.The improvement effect of soil matrix in highway spoil area decreases with the increase of the waste slag content,especially when the soil-slag ratio is less than 3,and the promotion of plants is limited.On the contrary,the improvement effect is proportional to the biochar content(3%-8%).But it is noted that the Cu and Pb in the soil will exceed the clean limit corresponding to the Nemero soil pollution index level when the biochar content is 8%.Therefore,it is recommended that the soil-slag ratio should be≥3,and the biochar content should reach 3%-5%.This research provides experimental basis and technical support for utilizing solid waste resources in the reclamation of highway spoil areas.

Multi-soliton solutions, breather-like and bound-state solitons for complex modified Korteweg–de Vries equation in optical fibers

Under investigation in this paper is a complex modified Korteweg–de Vries(KdV) equation, which describes the propagation of short pulses in optical fibers. Bilinear forms and multi-soliton solutions are obtained through the Hirota method and symbolic computation. Breather-like and bound-state solitons are constructed in which the signs of the imaginary parts of the complex wave numbers and the initial separations of the two parallel solitons are important factors for the interaction patterns. The periodic structures and position-induced phase shift of some solutions are introduced.