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Genotypes and Phenotypes of Uygur Children with Xeroderma Pigmentosum: A Case Study in Xinjiang, China

International journal of Dermatology and Venereology 2024年第3期7卷 144-149页

作者： Qiu Y. Wang W.-j. Liang j.-Q. Yakeya b. Wang P. Zhang j.-Z. Kang X.-j. Department of Dermatology and Venereology People's Hospital of Xinjiang Uygur Autonomous Region Xinjiang Uygur Autonomous Region Urumqi 830001 China Xinjiang Clinical Research Center for Dermatologic Diseases Xinjiang Uygur Autonomous Region Urumqi 830001 China Xinjiang Key Laboratory of Dermatology Research (XjYS1707) Xinjiang Uygur Autonomous Region Urumqi 830001 China

Objective: Xeroderma pigmentosum (XP) is a rare autosomal recessive dermatosis caused by genetic defects of DNA repair. This study was performed to detect and analyze the genes of 2 Uygur patients with XP and their families and assess the patients' phenotypes, which may enrich the understanding of the genetic skin disorder spectrum in Xinjiang area. Methods: We collected the clinical data from 2 patients with XP and peripheral blood samples from the patients and their family members. The patients' DNA was sequenced and detected by Sanger sequencing, and gene mutations were screened. Results: The proband in family 1 presented with brown maculae at the exposure site and squamous cell carcinoma secondary to a facial rash. The proband had a homozygous nucleotide variation of XPC c.2251-2A> G (A change from A to G in the penultimate position of the intron before the 2251 position in the coding region), which was a shear mutation. In this family, both parents were heterozygous, and no similar mutation detected in the sister. In family 2, the proband had scattered black brown spots and papules on the trunk and limbs. and his younger sister was also a patient. The proband and his younger sister had homonucleotide variation of XPA c.631C> T, which was nonsense mutation, resulting in the codon for Arg No.211 being changed into termination codon (***211X), thus terminating the peptide chain synthesis prematurely. All the normal individuals in the two families were heterozygotes, and homozygous mutations occurred in all the patients, which was consistent with the autosomal recessive inheritance. Conclusion: XP is rare in Uygur population. This study expanded the mutation spectrum of XP and provided a basis for early diagnosis, treatment, prognostic prediction, and prenatal genetic consultation. © 2022 Hospital for Skin Diseases (Institute of Dermatology), Chinese Academy of Medical Sciences, and Chinese Medical Association, published by Wolters Kluwer, Inc.

关键词： gene mutation xeroderma pigmentosum XPA gene XPC gene

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Effect of Al_(2)O_(3)on the Mechanical Properties of(b_(4)C+Al_(2)O_(3))/Al Neutron Absorbing Materials

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Acta Metallurgica Sinica(English Letters) 2024年第8期37卷 1411-1420页

作者： j.X.Cai b.M.Shi N.Li Y.Liu Z.G.Zhang Y.N.Zan Q.Z.Wang b.L.Xiao Z.Y.Ma Shi‑Changxu Innovation Center for Advanced Materials Institute of Metal ResearchChinese Academy of SciencesShenyang 110016China Key Laboratory of Nuclear Materials and Safety Assessment Institute of Metal ResearchChinese Academy of SciencesShenyang 110016China Nuclear Equipment Division Beijing Institute of Nuclear EngineeringChina Nuclear Power Engineering Co.Ltd.Beijing 100840China Department of Materials Science School of Mechanical EngineeringShenyang UniversityShenyang 110044China

b_(4)C/Al composites are widely utilized as neutron absorbing materials for the storage and transportation of spent nuclear *** order to improve the high-temperature mechanical properties of b_(4)C/Al composites,in-situ nano-Al_(2)O_(3)was introduced utilizing oxide on Al powder *** this study,the Al_(2)O_(3)content was adjusted by utilizing spheroid Al powder with varying diameters,thereby investigating the impact of Al_(2)O_(3)content on the tensile properties of(b_(4)C+Al_(2)O_(3))/Al *** was found that the pinning effect of Al_(2)O_(3)on the grain boundaries could hinder the recovery of dislocations and lead to dislocation accumulation at high *** the result,with the increase in Al_(2)O_(3)content and the decrease in grain size,the high-temperature strength of the composites increased *** finest Al powder used in this investigation had a diameter of 1.4μm,whereas the resultant composite exhibited a maximum strength of 251 MPa at room temperature and 133 MPa at 350℃,surpassing that of traditional b_(4)C/Al composites.

关键词： Al matrix composites Neutron absorbing materials (b_(4)C+Al_(2)O_(3))/Al composites High-temperature strength

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Thermochemical co-conversion of biomass-plastic waste to biochar:a review

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Green Chemical Engineering 2024年第1期5卷 31-49页

作者： Adewale George Adeniyi Kingsley O.Iwuozor Ebuka Chizitere Emenike Oluwaseun j.Ajala Samuel Ogunniyi Kabir b.Muritala Department of Chemical Engineering Faculty of Engineering and TechnologyUniversity of IlorinIlorinP.M.B.1515Nigeria Department of Chemical Engineering Landmark UniversityOmu-AranKwara StateP.M.B.1001Nigeria Department of Pure and Industrial Chemistry Nnamdi Azikiwe UniversityAwkaP.M.B.5025Nigeria Department of Chemistry Federal University of TechnologyBosso CampusMinnaNiger StateP.M.B.65Nigeria

biomass and plastics are two of the most common municipal solid wastes globally that have continuously placed a burden on the *** is therefore important that they are properly *** coconversion offers a valuable opportunity to recycle biomass and plastics simultaneously into biochar,which reduces the time and cost of recycling them individually while producing a material with a wide range of *** study is a review of published literature that discusses the thermochemical co-processing of biomass and plastic wastes into *** was observed that co-pyrolysis and co-hydrothermal carbonization are the most commonly utilized technologies for this *** characteristics of different biomass and plastics that have been thermochemically converted into biochar were *** properties of the resulting biochar are affected by the feedstock composition,pre-treatment and blending ratio,the reactor’s configuration,reaction temperature,and the presence of a *** studies found that treating the feedstocks separately resulted in a lower yield of biochar than processing them *** biochar created by this procedure has been used as a soil additive and as an adsorbent for water *** perspectives and suggestions,such as the necessity for some technical advancement,biochar’s economic benefits,improved government participation,and raised social awareness,were also *** factors have the potential to propel this field of study to great horizons.

关键词： biomass Co-pyrolysis Plastic Thermochemical process Valorization

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Optimization of chemistry and process parameters for control of intermetallic formation in Mg sludges

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journal of Magnesium and Alloys 2024年第4期12卷 1431-1448页

作者： Y.Fu G.G.Wang A.Hu Y.Li K.b.Thacker j.P.Weiler H.Hu Department of Mechanical Automotive and Materials EngineeringUniversity of Windsor401 Sunset AveWindsorOntario N9B 3P4Canada Global Technology Centre Meridian Lightweight Technologies25 MacNab AveStrathroyOntario N7G 4H6Canada

Intermetallic formation in sludge during magnesium(Mg)melting,holding and high pressure die casting practices is a very important ***,very often it is overlooked by academia,original equipment manufacturers(OEM),metal ingot producers and even die *** aim of this study was to minimize the intermetallic formation in Mg sludge via the optimization of the chemistry and process *** Al8Mn5 intermetallic particles were identified by the microstructure analysis based on the Al and Mn *** design of experiment(DOE)technique,Taguchi method,was employed to minimize the intermetallic formation in the sludge of Mg alloys with various chemical compositions of Al,Mn,Fe,and different process parameters,holding temperature and holding *** sludge yield(SY)and intermetallic size(IS)was selected as two *** optimum combination of the levels in terms of minimizing the intermetallic formation were 9 wt.%Al,0.15 wt.%Mn,0.001 wt.%(10 ppm)Fe,690℃ for the holding temperature and holding at 30 mins for the holding time,*** best combination for smallest intermetallic size were 9 wt.%Al,0.15 wt.%Mn,0.001 wt.%(10 ppm)Fe,630℃ for the holding temperature and holding at 60 mins for the holding time,*** groups of sludge factors,Chemical Sludge(CSF),Physical Sludge(PSF)and Comprehensive Sludge Factors(and CPSF)were established for prediction of sludge yields and intermetallic sizes in Al-containing Mg *** CPSF with five independent variables including both chemical elements and process parameters gave high accuracy in prediction,as the prediction of the PSF with only the two processing parameters of the melt holding temperature and time showed a relatively large deviation from the experimental *** Chemical Sludge Factor was primarily designed for small ingot producers and die casters with a limited melting and holding capacity,of which process parameters could be fixed *** Physical Sludge Factor could be used f

关键词： Magnesium sludge Al-Mn intermetallic Optimization Taguchi method Sludge factor Chemical composition Process parameter

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Atomistic Investigation of Shock-Induced Amorphization within Micro-shear bands in Hexagonal Close-Packed Titanium

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Acta Metallurgica Sinica(English Letters) 2024年第9期37卷 1590-1600页

作者： Z.C.Meng K.G.Wang T.Ali D.Li C.G.bai D.S.Xu S.j.Li A.H.Feng G.j.Cao j.H.Yao Q.b.Fan H.Wang R.Yang Institute of Metal Research Chinese Academy of SciencesShenyang 110016China School of Materials Science and Engineering University of Science and Technology of ChinaShenyang 110016China Interdisciplinary Centre for Additive Manufacturing(ICAM) School of Materials and ChemistryUniversity of Shanghai for Science and TechnologyShanghai 200093China School of Materials Science and Engineering Tongji UniversityShanghai 201804China Key Laboratory for Light-Weight Materials Nanjing Tech UniversityNanjing 210009China National Key Laboratory of Science and Technology on Materials Under Shock and Impact School of Materials Science and EngineeringBeijing Institute of TechnologyBeijing 100081China beijing Institute of Technology Chongqing Innovation Center Chongqing 401135China

The mechanical response of a single crystal titanium sample against(0001)α surface impact was investigated using molecular dynamics ***,non-uniform plastic deformation was observed in the *** high strain rates,amorphization occurred near the edge of the contact region where severe shear strain induced a large number of stacking faults(SFs)and *** contrast,the central part of the contact region underwent less deformation with significantly fewer ***,instead of amorphization by consuming SFs and dislocations,there was a gradual increase in the density of dislocations and SFs during the process of *** local amorphous regions eventually grew into shear bands.

关键词： Shock compression Amorphization Shear band Titanium Molecular dynamics

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Comparative utility of vestibular function tests in patients with peripheral and central vestibular dysfunction

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journal of Otology 2024年第1期19卷 5-9页

作者： Cameron b.Fattahi Christopher Zaro janice j.Chung Richard F.Lewis Divya A.Chari Department of Otolaryngology-Head and Neck Surgery UMASS Memorial Medical CenterUniversity of Massachusetts Chan Medical SchoolWorcesterMAUSA jenks Vestibular Physiology Laboratory Massachusetts Eye and EarBostonMAUSA Department of Otolaryngology-Head and Neck Surgery Massachusetts Eye and EarHarvard Medical SchoolBostonMAUSA Department of Neurology Harvard Medical SchoolBostonMAUSA

background: bithermal caloric irrigation, video head impulse test(vHIT), and rotational testing are commonly used to assess peripheral vestibular function, but the relative clinical utility of each test in differentiating patients with peripheral vestibulopathy is ***: To determine whether(1) the combination of two or more vestibular tests enhances diagnostic utility over a single test;(2) abnormal test results on vestibular tests correlate with one ***: Retrospective analysis of data collected from multidisciplinary vestibular clinics at two academic medical centers from 2016 to ***: 150 patients(54.10 ± 15.09 years, 88 females) were included. No individual test was significantly better at predicting the presence of peripheral vestibular damage(p > 0.05). vHIT test results improved significantly when combined with either the caloric test(p = 0.007) or rotary chair test(p = 0.039). Caloric and rotational testing had high sensitivity(74.65% and 76.06%, respectively) and specificity(83.54% and 78.48%, respectively). vHIT demonstrated excellent specificity(89.87%) but poor sensitivity(47.89%). Caloric, vHIT, and rotary chair tests results did not correlate with one another(p > 0.05).Conclusions: Vestibular function tests have comparable diagnostic utility, yet each offers unique advantages. Caloric and rotational testing may be best suited for screening peripheral damage and vHIT may function ideally as a confirmatory test.

关键词： vestibular patients specificity

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A facile strategy for tuning the density of surface-grafted biomolecules for melt extrusion-based additive manufacturing applications

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bio-Design and Manufacturing 2024年第3期7卷 277-291页

作者： I.A.O.beeren G.Dos Santos P.j.Dijkstra C.Mota j.bauer H.Ferreira Rui L.Reis N.Neves S.Camarero-Espinosa M.b.baker L.Moroni Department of Complex Tissue Regeneration MERLN Institute for Technology-Inspired Regenerative MedicineMaastricht University6229 ER MaastrichtThe Netherlands 3b’s Research Group I3Bs–Research Institute on BiomaterialsBiodegradables and BiomimeticsHeadquarters of the European Institute of Excellence on Tissue Engineering and Regenerative MedicineUniversity of MinhoAveParkZona Industrial da Gandra4805-017 BarcoGuimarãesPortugal ICVS/3b’s-PT Government Associate Laboratory 4806-909 Braga/GuimarãesPortugal POLYMAT University of the Basque Country UPV/EHU20018 Donostia/San SebastiánSpain IKERbASQUE Basque Foundation for Science48009 BilbaoSpain

Melt extrusion-based additive manufacturing(ME-AM)is a promising technique to fabricate porous scaffolds for tissue engi-neering ***,most synthetic semicrystalline polymers do not possess the intrinsic biological activity required to control cell *** of biomolecules on polymeric surfaces of AM scaffolds enhances the bioactivity of a construct;however,there are limited strategies available to control the surface ***,we report a strategy to tune the surface density of bioactive groups by blending a low molecular weight poly(ε-caprolactone)5k(PCL5k)containing orthogonally reactive azide groups with an unfunctionalized high molecular weight PCL75k at different *** porous three-dimensional(3D)scaf-folds were then fabricated using a high weight percentage(75 wt.%)of the low molecular weight PCL *** a proof-of-concept test,we prepared films of three different mass ratios of low and high molecular weight polymers with a thermopress and reacted with an alkynated fluorescent model compound on the surface,yielding a density of 201-561 pmol/cm^(2).Subsequently,a bone morphogenetic protein 2(bMP-2)-derived peptide was grafted onto the films comprising different blend compositions,and the effect of peptide surface density on the osteogenic differentiation of human mesenchymal stromal cells(hMSCs)was *** two weeks of culturing in a basic medium,cells expressed higher levels of bMP receptor II(bMPRII)on films with the conjugated *** addition,we found that alkaline phosphatase activity was only significantly enhanced on films contain-ing the highest peptide density(i.e.,561 pmol/cm^(2)),indicating the importance of the surface *** together,these results emphasize that the density of surface peptides on cell differentiation must be considered at the cell-material ***,we have presented a viable strategy for ME-AM community that desires to tune the bulk and surface functionality via blending of(m

关键词： Additive manufacturing blending Surface functionalization Surface density Click chemistry Human

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Investigating ischemia and reperfusion-induced organ damage in severe cardiac arrest:A comprehensive proteomics perspective

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journal of Pharmaceutical Analysis 2024年第3期14卷 427-430页

作者： ju Yeon Lee Muhammad Shoaib jin-Woong Choi Rishabh C.Choudhary Tai Yin Nara Yoon Kei Hayashida Seunguk j.baek Santiago j.Miyara Lance b.becker junhwan Kim Research Center of bioconvergence Analysis Korea Basic Science InstituteCheongjuSouth Korea bio-Analytical Science University of Science and TechnologyCheongjuSouth Korea Laboratory for Critical Care Physiology Feinstein Institutes for Medical ResearchManhassetNY11030USA Donald and barbara Zucker School of Medicine at Hofstra/Northwell School of Medicine HempsteadNY11549USA Mathematics and Computer Science Adelphi UniversityGarden CityNY11530USA Department of Emergency Medicine North Shore University HospitalManhassetNY11030USA

Cardiac arrest(CA)is a life-threatening condition with complex pathophysiology and limited treatment *** gain deeper insights into the pathological state of vital organs,we employed a proteomics analysis in rodents to assess proteome alterations in the brain,heart,kidney,and liver using a rat model of *** brain displayed severe protein alterations in essential cellular pathways,including three major energy-generating pathways after CA,which worsened after resuscitation,resulting in the most significant overall protein changes among the ***,the liver,experiencing the most substantial protein alterations post-CA,demonstrated significant recovery,presenting the least protein changes post-resuscitation.

关键词： alterations organs damage

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Complexed amino acid minerals ***-glycinate chelated minerals:Impact on the performance of old laying hens

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Animal Nutrition 2024年第1期16卷 395-408页

作者： Marcos j.b.Santos Maria C.M.M.Ludke Leandro M.Silva Carlos b.V.Rabello Mércia R.barros Fabiano S.Costa Clariana S.Santos jamille S.S.Wanderley Department of Animal Science Rural Federal University of PernambucoRecifePEBrazil Department of Veterinary Science Rural Federal University of PernambucoRecifePEBrazil

The present study was to evaluate the effect of trace minerals(Zn,Mn,and Cu)from complexed amino acid minerals(ZMCAA)and bis-glycinate chelated minerals(ZMCGly)in laying hen diets on performance,internal and external egg quality,yolk mineral deposition,intestinal morphometry,and bone *** 78 to 98 weeks of age,400 White LSL-Lite strain laying hens were distributed in a randomized design with 4 treatments with 10 replicates per *** were distributed in a 2×2 factorial arrangement using either Zn,Mn,and Cu of ZMCAA or ZMCGly source at 2 levels:low(20,20,and 3.5 mg/kg of Zn,Mn,and Cu,respectively)or high(40,40,and 7 mg/kg of Zn,Mn,and Cu,respectively).The analysis of variance was performed,and in cases where differences were observed,the means were compared using Tukey?s test(Pbumen height(P=0.01),albumen weight(P=0.01),and eggshell thickness(Pbone densitometry(Pbia were higher with ZMCAA *** the small intestine,ZMCAA resulted in longer villi(P=0.02)and shorter crypt depth(P=0.01)in the *** and ileum measurements were influenced by the level and source of trace minerals(Pbited superior performance,egg quality,deposition of trace minerals in the egg yolk,and bone density compared to hens fed *** this study,older laying hens supplemented with ZMCAA at lower levels demonstrated adequate levels of supplementation.

关键词： bis-glycinates Metal-amino acids Radiodensity Trace minerals

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Current Zoology 2024年第1期70卷 1-12页

作者： Lucia V.Sommaro juan j.Martinez Marina b.Chiappero Andrea R.Steinmann Cristina N.Gardenal joséW.Priotto Laboratorio de Ecologia Evolutiva y biogeografia Instituto de Ecorregiones Andinas(INECoA)Universidad Nacional de Jujuy-Consejo Nacional de Investigaciones Cientificas y Tecnicas(CONICET)San Salvador de JujuyJujuyArgentina Instituto de Diversidad y Ecologia Animal(IDEA) Consejo Nacional de Investigaciones Cientificas y Técnicas(CONICET)and Universidad Nacionalde CordobaCordobaArgentina Catedra de Genética de Poblaciones y Evolucion Facultad de Ciencias ExactasFisicas y NaturalesUniversidad Nacional de CordobaCordobaArgentina Grupo de Investigaciones en Ecologia Poblacional y Comportamental(GIEPCO) Instituto de Ciencias de la TierraBiodiversidad y Ambiente(ICBiA)Universidad Nacional de Rio Cuarto-Consejo Nacional de Investigaciones Cientificas y Técnicas(CONICET)Rio CuartoCordobaArgentina

The sex-biased dispersal and kinship dynamics are important factors shaping the spatial distribution of individuals and are key parameters affecting a variety of ecological and evolutionary ***,we studied the spatial distribution of related individuals within a population of corn mice Calomys musculinus in a seasonal cycle to infer dispersal *** sampling was carried out from spring 2005 to winter 2006 in field borders of intensively managed *** data from 346 individuals with 9 microsatellites showed spatial genetic structure was weak for males,but not for *** results indicate a complex spatial kinship dynamic of related females across all ***,contrary to our expectations,dispersal distances decrease with the increase of the population ***,male dispersal distances were greater when population abundance increased and thus the availability of active *** disperse greater distances to mate and sire offspring with distant females as a possible inbreeding avoidance *** study shows that *** is capable of much greater scattering distances than previously reported and that dispersal occurs fluidly and without barriers across the *** indirect benefit of dispersal on individual fitness could be related to relaxing the competition in the natal area and increasing the mating *** study highlights the value of combining genetic relatedness,fieldwork observations,and behavioral data to estimate dispersal at a fine geographical scale.

关键词： dispersal distances kinship dynamics microsatellite small mammals spatial autocorrelation

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