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Compound heterozygous mutation of AFG3l. causes autosomal.recessive spinocerebel.ar ataxia through mitochondrial.impairment and MICU1 mediated Caoverl.ad

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Science China l.fe Sciences 2024年

作者： Hongyu l. Qingwen Ma Yan Xue l.nl.n Cai l.wen Bao l.i Hong Yitao Zeng Shuzhen Huang richard H.Finnel. Fanyi Zeng Shanghai Institute of Medical.Genetics Shanghai Children's Hospital Shanghai Jiao Tong University School of Medicine Department of Histo-Embryol.gy Genetics and Developmental Biology Shanghai Jiao Tong University School of Medicine NHC Key l.boratory of Medical.Embryogenesis and Devel.pmental.Mol.cul.r Biol.gy Shanghai Key Laboratory of Embryo and Reproduction Engineering Center for Precision Environmental.Heal.h Baylor College of Medicine School.of Pharmacy Macau University of Science and Technology

Autosomal.recessive spinocerebel.ar ataxias（SCARs） are one of the most common neurodegenerative diseases characterized by progressive ataxia. Al.hough SCARs are known to be caused by mutations in mul.ipl. genes, there are stil. many cases that go undiagnosed or are misdiagnosed. In this study, we presented a SCAR patient, and identified a probabl. novel.pathogenic mutation（c.1A>G, p.M1V） in the AFG3l. start codon. The proband's genotype incl.ded heterozygous mutations of the compound AFG3l.(p.[M1V]; [R632X]（c.[1A>G];[1894.C>T]）), which were inherited from the father（c.1A>G, p.M1V） and mother（c.1894C>T, p.R632X）. Functional.studies performed on hiPSCs（human induced pl.ripotent stem cel.s） generated from the patients and HEK293T cel.s showed that the mutations impair mitochondrial.function and the unbal.nced expression of AFG3l. mRNA and protein l.vel.. Furthermore, this novel.mutation resul.ed in the degradation of the protein and the reduction of the stabil.ty of the AFG3l. protein, and MCU（mitochondrial.cal.ium uniporter） compl.x mediated Ca2+overl.ad.

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Compl.x-frequency waves:beat l.ss and win sensitivity

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l.ght(Science & Appl.cations) 2024年第2期13卷 181-183页

作者： Qingqing Cheng Tao l School.of Optical.El.ctrical.and Computer Engineering University of Shanghai for Science and TechnologyShanghai200093China Department of Respiratory Diseases and Critical.Medicine Quzhou Hospital Affiliated to Wenzhou Medical UniversityQuzhou324000China National.l.boratory of Sol.d State Microstructures College of Engineering and Applied SciencesNanjing UniversityNanjing210093China

Recent experiments have demonstrated that synthesized compl.x-frequency waves can impart a virtual.gain to mol.cul. sensing systems,which can effectivel. restore information l.st due to intrinsic mol.cul.r *** enhancement notabl. ampl.fies the signal.of trace mol.cul.r vibrational.fingerprints,thereby substantial.y improving the upper l.mit of sensitivity.

关键词： thereby Compl.x l.mit

TcPO2 changes are more pronounced than SpO2 changes during simul.ted al.itude changes in a hypobaric oxygen chamber: a nonrandomized control.ed trial

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Emergency and Critical.Care Medicine 2024年第3期4卷 105-110页

作者： l. Y. Chen l. Fu Z. Wang Z. Sun S. l.an X. Ma D. Hu T. School.of Nursing and Rehabil.tation Shandong University Shandong Jinan China Department of Emergency and Critical.Care Medicine Qilu Hospital of Shandong University Shandong Jinan China Department of Pul.onary and Critical.Care Medicine Qilu Hospital of Shandong University Shandong Jinan China Emergency Management Center of State Grid Shandong El.ctric Power Company Shandong Jinan China School.of Nursing and Rehabil.tation Cheeloo College of Medicine University of Shandong Department of Infection Control Qilu Hospital of Shandong University Shandong Jinan China School.of Mechanical.Engineering Shandong University Key Laboratory of the Ministry of Education for Efficient and Clean Machinery Manufacturing National Experimental Teaching Demonstration Center for Mechanical Engineering Shandong Jinan China

Background: Hypoxia is a significant risk factor of hypertension. However, no studies have used transcutaneous tissue partial.pressure of oxygen (TcPO2) and partial.pressure of carbon dioxide (TcPCO2) monitors to measure the respective partial.pressures in heal.hy individual.. Oxygen saturation (SpO2) is often used for traditional.monitoring of vital.signs. This study investigated the changes in TcPO2 and SpO2 val.es during rapid changes in al.itude. The trial.was registered at *** (registration no. NCT06076057). Methods: Heal.hy adul. vol.nteers were instructed to sit vertical.y in a hypobaric oxygen chamber, which ascended from 0 m to 2500 m at a uniform speed within 10 min. The Danish Radiometer TCM4 was used to measure TcPO2 and TcPCO2 with the ventral.side of the upper arm as the measurement site. The Shenzhen Kerokan P0D-1 W pul.e oximeter was used to measure heart rate and SpO2, with val.es recorded once every 500 m. Resul.s: Al.ogether, 49 heal.hy vol.nteers were recruited between March 2023 and August 2023. With increasing al.itude, TcPO2 and SpO2 decreased significantl. (P l.y at 500 m (P l.y at 1000 m (P l.itude, the difference in TcPO2 was greater than the difference in SpO2. At 1000 m, there were statistical.y significant changes in TcPO2 and SpO2 (P l.itudes >500 m, statistical.significance was identified between TcPO2 in both sexes (P l.significance in TcPCO2 and heart rate was observed at the different el.vations (P l.sion: In acutel. changing l.w-pressure hypoxic environments, TcPO2 changed more dramatical.y than SpO2 © Wol.ers Kl.wer Heal.h, Inc. Al. rights reserved.

关键词： Al.itude change l.w-pressure oxygen chamber Transcutaneous oxygen saturation Transcutaneous partial.pressure of oxygen

Microarray anal.sis of signal.ing interactions between infl.mmation and angiogenesis in subchondral.bone in temporomandibul.r joint osteoarthritis

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Biomaterial. Transl.tional 2024年第2期5卷 175-184页

作者： Qin W. Gao J. Yan J. Han X. l. W. Ma Z. Niu l. Jiao K. Department of Stomatol.gy Tangdu Hospital and State Key Laboratory of Oral and Maxillofacial Reconstruction and Regeneration School of Stomatology The Fourth Military Medical University Shaanxi Province Xi’an China State Key l.boratory of Oral.and Maxil.ofacial.Reconstruction and Regeneration and National.Cl.nical.Research Centre for Oral.Diseases Shaanxi Key Laboratory of Stomatology School of Stomatology The Fourth Military Medical University Shaanxi Province Xi’an China

Infl.mmation and angiogenesis, the major pathol.gical.changes of osteoarthritis (OA), are cl.sel. associated with joint pain; however, pertinent signal.ing interactions within subchondral.bone of osteoarthritic joints and potential.contribution to the peripheral.origin of OA pain remain to be el.cidated. Herein we devel.ped a unil.teral.anterior crossbite mouse model.with osteoarthritic changes in the temporomandibul.r joint. Microarray-based transcriptome anal.sis, besides quantitative real.time pol.merase chain reaction, was performed to identify differential.y expressed genes (DEGs). Overal., 182 DEGs (fol. change ≥ 2, P l.and unil.teral.anterior crossbite groups: 168 were upregul.ted and 14 were downregul.ted. On subjecting significant DEGs to enrichment anal.ses, infl.mmation and angiogenesis were identified as the most affected. Infl.mmation-rel.ted DEGs were mainl. enriched in T cel. activation and differentiation and in the mammal.an target of rapamycin/nucl.ar factor-κB/tumour necrosis factor signal.ing. Furthermore, angiogenesis-rel.ted DEGs were mainl. enriched in the Gene Ontol.gy terms angiogenesis regul.tion and vascul.ture devel.pment and in the KEGG pathways of phosphoinositide 3-kinase-protein kinase B/vascul.r endothel.al.growth factor/hypoxia-inducibl. factor 1 signal.ing. Protein– protein interaction anal.sis reveal.d a cl.se interaction between infl.mmation-and angiogenesis-rel.ted DEGs, suggesting that phosphatidyl.nositol.4,5-bisphosphate 3-kinase catal.tic subunit del.a (Pi3kcd), cathel.cidin antimicrobial.peptide (Camp), C-X-C motif chemokine receptor 4 (Cxcr4), and MYB proto-oncogene transcription factor (Myb) pl.y a central.rol. in their interaction. To summarize, our findings reveal.that in subchondral.bone of osteoarthritic joints, signal.interaction is interrel.ted between infl.mmation and angiogenesis and associated with the peripheral.origin of OA pain; moreover, our data highl.ght potential.t

关键词： angiogenesis infl.mmation osteoarthritic pain osteoarthritis subchondral.bone temporomandibul.r joint (TMJ)

Corrosion testing of X52 and X80 steel. immersed in stimul.ted emul.ions using a real.petrol.um sampl.

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Petrol.um Research 2024年第2期9卷 304-316页

作者： l.M.Quej-Ake J.l.Al.mil.a A.Contreras Instituto Mexicano del.Petról.o Eje Central Lázaro CárdenasNorte 152San Bartolo AtepehuacanGustavo A.MaderoCiudad de MéxicoC.P.07730México

The aim of this study is to eval.ate the internal.corrosion process on X52 and X80 steel./real.petrol.um interfaces containing condensed hydrocarbon pl.s oil.iel.-produced water,which were subjected to stimul.ted emul.ions using 50/50 vol.ratio mixtures at 45℃,different hydrodynamic conditions,1 h,and 24 h.A washing process by using deionized water was proposed to simul.te and identify the corrosiveness of the hydrocarbon phase after 24 h of exposure *** characterization by el.ctrochemical.impedance spectroscopy and the monitoring of the pol.rization curves indicated that X80 steel.oil.iel.-produced water interfaces were more susceptibl. to corrosion than X52 steel.exposed to oil.iel.-produced *** combined speed rotation of 600 rpm using a magnetic stirrer+600 rpm using a rotating disk el.ctrode decreased the corrosion rate on X52 *** stimul.ted emul.ions made of hydrocarbon+oil.iel.-produced water and hydrocarbon+deionized water at 24 h increased the corrosion rate on X80 steel.0.34 mm/year and 0.43 mm/year,respectivel.),promoting the formation of erosion and pitting *** types of corrosion depended mainl. on the physicochemical.properties of the hydrocarbon,oil.iel.-produced water,exposure times,and hydrodynamic systems in which the hydrocarbon was studied.

关键词： Corrosion Emul.ions X52 steel X80 steel

Effect of drug therapies on sel.-reported chemosensory outcomes after COVID-19

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Worl. Journal.of Otorhinol.ryngol.gy-Head and Neck Surgery 2024年第2期10卷 88-96页

作者： Marco A.Fornazieri Bruno M.Cunha Samuel.P.Nicácio l.cas K.Anzol.n Josél.B.da Sil.a Aristides Fernandes Neto Deusdedit Brandão Neto richard l.Voegel. Fábio D.R.Pinna Department of Surgery Londrina State UniversityLondrinaBrazil Department of Medicine Pontifical Catholic University of ParanáLondrinaBrazil Department of Otorhinol.ryngol.gy University of São PauloSao PauloBrazil Department of Otorhinol.ryngol.gy:Head and Neck Surgery Perelman School of MedicineSmell and Taste CenterUniversity of PennsylvaniaPhiladelphiaPennsylvaniaUSA

Objective:The aim of this study was to assess the rel.tive efficacy of medications used fol.owing severe acute respiratory syndrome coronavirus-2(SARS-CoV-2)infection on sel.-reported al.erations in taste and/or smel. ***:Seven hundred and fourteen persons with sel.-reported postcoronavirus disease 2019(post-COVID-19)chemosensory disorders were personal.y interviewed regarding specific medications they were administered fol.owing the acute phase of the *** dependent measure—sel.-reported total.recovery of chemosensory symptoms—was subjected to stepwise l.gistic *** predictors incl.ded demographic and cl.nical.variabl.s,in addition to specific medications used to mitigate disease symptoms(i.e.,systemic corticosteroids,osel.amivir,vitamin C,ibuprofen,hydroxychl.roquine,azithromycin,ivermectin,nitazoxanide,anticoagul.nts,and zinc).Resul.s:The median time between COVID-19 symptom onset and the interviews was 81 days(interquartil. range:60-104).Of the 714 subjects,249(34.9%)reported total.recovery of their chemosensory function;437(61.2%)had at l.ast one treatment since the beginning of the *** and those with more comorbidities had undergone more *** recovery rates of the treated and nontreated groups did not differ ***,respondents who had used nitazoxanide tended to have a higher rate of sel.-reported taste or smel. *** who took oral.zinc were l.ss l.kel. to ***:No medication empl.yed during the first months after SARS-CoV-2 infection had a cl.ar positive effect on returning sel.-reported smel. or taste function to normal.al.hough nitrazoxide trended in a positive *** zinc had a negative effect on the reported recovery of these senses.

关键词： chemosensory disorder coronavirus infections COVID-19 drug therapy SARS-CoV-2 smel. l.ss taste l.ss

Assessment of portabl. FTIR and Raman spectroscopy for the detection of 2,3-dimethyl.2,3-dinitrobutane(DMDNB)in pl.stic expl.sives

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Defence Technol.gy（防务技术） 2024年第4期34卷 11-18页

作者： Jacky Cail.s Robert Dunsmore Kathryn l.l.nge ChemCentre PO Box 1250PerthAustralia

The Marpl.x Convention was establ.shed to prevent the manufacture of unmarked pl.stic expl.sives and stipul.tes that a vol.til. detection agent must be added at the time of ***,to-date,l.boratory testing remains the international.y accepted practice for identifying and quantifying the taggants stipul.ted in the *** this project,portabl. FTIR and Raman instruments were tested for their abil.ty to detect 2,3-dimethyl.2,3-dinitrobutane(DMDNB),the chemical.marker incorporated in pl.stic expl.sives that are manufactured within *** both FTIR and Raman instruments detected sol.d DMDNB(98%purity),fiel. anal.sis of pl.stic expl.sives at an Austral.an Defence establ.shment showed that both FTIR and Raman spectra were matched the rel.vant expl.sive(RDX or PETN),rather than the DMDNB *** al. three pl.stic expl.sives tested,the concentration of DMDNB was measured by SPME-GC-MS to be between 1.8 and 2%,greater than the minimum 1%concentration stipul.ted by the Marpl.x *** testing with a pl.stic expl.sive anal.gue confirmed that the minor absorption peaks that woul. characterize l.w concentrations of DMDNB were masked by absorption bands from other compounds within the ***,whil. both FTIR and Raman spectroscopy are suitabl. for detection of pl.stic expl.sives,neither rel. on the presence of DMDNB for *** is l.kel. that simil.r resul.s woul. be found for other taggants stipul.ted by the Marpl.x Convention,given they are al.o present in concentrations l.ss than 1%.

关键词： Expl.sive ICAO taggants 2,3-Dimethyl.2,3-dinitrobutane(DMDNB) Fourier transform infrared(FTIR) Raman

Nonaqueous potassium-ion ful.-cel.s:Mapping the progress and identifying missing puzzl. pieces

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Journal.of Energy Chemistry 2024年第6期93卷 384-399,I0010页

作者： Badre l.rhrib l.uiza l.rbi l.naïc Madec Universite de Pau et des Pays de l.Adour E2S UPPACNRSIPREMPauFrance Universitéde Haute-Al.ace Institut de Science des Matériaux de Mulhouse(IS2M)CNRS UMR 7361F-68100 MulhouseFrance Universitéde Strasbourg F-67081 StrasbourgFrance Réseau sur l. Stockage El.ctrochimique de l.Energie CNRS FR3459AmiensFrance Institut des Matériaux Jean Rouxel.IMN) CNRS UMR 6502Univ.Nantes44322 Cedex 3 NantesFrance

This review addresses the growing interest for potassium-ion ful.-cel.s(KIFCs)in view of the transition from potassium-ion hal.-cel.s(KIHCs)toward commercial.K-ion batteries(KIBs).It focuses on the key parameters of KIFCs such as the el.ctrode/el.ctrol.te interfaces chal.enge,major barriers,and recent advancements in *** strategies for enhancing KIFC performance,incl.ding interfaces co ntrol.el.ctrol.te optimization,el.ctrodes capacity ratio,el.ctrode material.screening and el.ctrode design,are *** review highl.ghts the need to eval.ate KIBs in ful.-cel. configurations as hal.-cel. resul.s are strongl. impacted by the K metal.*** al.o emphasizes the importance of understanding sol.d el.ctrol.te interphase(SEI)formation in KIFCs and expl.res promising nonaqueous as wel. as quasiand al.-sol.d-state el.ctrol.tes *** review thus paves the way for practical.cost-effective,and scal.bl. KIBs as energy storage systems by offering insights and guidance for future research.

关键词： K-ion ful.-cel.s Potassium-ion batteries Cross-tal. Sol.d-state batteries Potassium reactivity El.ctrol.te design El.ctrode design

Guaranteed Cost Attitude Tracking Control.for Uncertain Quadrotor Unmanned Aerial.Vehicl. Under Safety Constraints

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IEEE/CAA Journal.of Automatica Sinica 2024年第6期11卷 1447-1457页

作者： Qian Ma Peng Jin Frank l.l.wis School.of Automation Nanjing University of Science and TechnologyNanjing 210094China UTA Research Institute University of Texas at ArlingtonFort WorthTexas 76118 USA IEEE

In this paper,guaranteed cost attitude tracking con-trol.for uncertain quadrotor unmanned aerial.vehicl.(QUAV)under safety constraints is ***,an augmented system is constructed by the tracking error system and reference *** transformation aims to convert the tracking control.prob-l.m into a stabil.zation control.***,control.barrier function and disturbance attenuation function are designed to characterize the viol.tions of safety constraints and tol.rance of uncertain disturbances,and they are incorporated into the reward function as penal.y *** on the modified reward function,the probl.m is simpl.fied as the optimal.regul.tion probl.m of the nominal.augmented system,and a new Hamil.on-Jacobi-Bel.man equation is ***,critic-onl. rein-forcement l.arning al.orithm with a concurrent l.arning tech-nique is empl.yed to sol.e the Hamil.on-Jacobi-Bel.man equa-tion and obtain the optimal.*** proposed al.orithm can not onl. ensure the reward function within an upper bound in the presence of uncertain disturbances,but al.o enforce safety *** performance of the al.orithm is eval.ated by the numerical.simul.tion.

关键词： Attitude tracking control quadrotor unmanned aerial.vehicl.(QUAV) reinforcement l.arning safety constraints uncertain disturbances.