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Biochemical consequences of glucocerebrosidase 1 mutations in Parkinson’s disease

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Neural Regeneration Research 2024年第4期19卷 725-727页

作者： Jeong hyun yoon Chiao-Yin Lee Anthony HV Schapira Department of Clinical and Movement Neurosciences University College London Institute of Neurology Faculty of Medicine Imperial College London Aligning Science Across Parkinson's(ASAP)Collaborative Research Network

Parkinson’s disease(PD,OMIM#168600)is a common neurodegenerative disorder with a global prevalence of approximately 8.5 *** is characterized by four cardinal motor symptoms:bradykinesia,rigidity,resting tremor,and subsequently by postural *** usually involves non-motor symptoms such as rapid eye movement sleep disorder,dementia,anosmia,and autonomic *** gene glucocerebrosidase 1(GBA1),which encodes the lysosomal enzyme glucocerebrosidase(GCase)(IUBMB:EC 3.***.1.45),shows strong linkage with PD;variants of GBA1 are the commonest genetic association with PD(Sidransky et al.,2009).Several mechanisms may underlie the relationship between GBA1 mutations/variants and the molecular pathology of PD(Figure 1A and B).

关键词： cerebro consequences

Long-term outcomes of very low birth weight infants with intraventricular hemorrhage:a nationwide population study from 2011 to 2019

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World Journal of Pediatrics 2024年第7期20卷 692-700页

作者： Joonsik Park sook-hyun Park Yu-ra Kwon So Jin yoon Joo Hee Lim Jung Ho Han Jeong Eun Shin Ho Seon Eun Min Soo Park Soon Min Lee Department of Pediatrics Yonsei University College of Medicine211 Eonjuro GangnamguSeoul 06273Republic of Korea

Background Advancements in neonatal care have increased preterm infant survival but paradoxically raised intraventricular hemorrhage(IVH)*** study explores IVH prevalence and long-term outcomes of very low birth weight(VLBW)infants in Korea over a *** Using Korean National Health Insurance data(NHIS,2010-2019),we identified 3372 VLBW infants with IVH among 4,129,808 live ***-related claims data,encompassing diagnostic codes,diagnostic test costs,and administered procedures were sourced from the NHIS *** results of the developmental assessments are categorized into four groups based on standard deviation(SD)*** characteristics and complications were compared among the *** regression models were employed to identify significant changes in the incidence of complications and to calculate odds ratios with corresponding 95%confidence intervals for each risk factor associated with mortality and morbidity in ***-term growth and development were compared between the two groups(years 2010-2013 and 2014-2017).Results IVH prevalence was 12%in VLBW and 16%in extremely low birth weight(ELBW)*** the past decade,IVH rates increased significantly in ELBW infants(P=0.0113),while mortality decreased(P=0.0225).Major improvements in certain neurodevelopmental outcomes and reductions in early morbidities have been observed among VLBW infants with *** percent of the population received surgical treatments such as external ventricular drainage(EVD)or a ventriculoperitoneal(VP)shunt,with the choice of treatment methods remaining consistent over *** IVH with surgical intervention group exhibited higher incidences of delayed development,cerebral palsy,seizure disorder,and growth failure(height,weight,and head circumference)up to 72 months of age(P<0.0001).Surgical treatments were also significantly associated with abnormal developmental screening test *** The neurodevelopmental outcomes of infa

关键词： Cerebral intraventricular hemorrhage Developmental Hydrocephalus Mortality Posthemorrhagic ventricular dilatation Surgery

Effects of Initial and Boundary Conditions on Heavy Rainfall Simulation over the Yellow Sea and the Korean Peninsula:Comparison of ECMWF and NCEP Analysis Data Effects and Verification with Dropsonde Observation

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Advances in Atmospheric Sciences 2024年第9期41卷 1787-1803页

作者： Jiwon HWANG Dong-hyun CHA Donghyuck yoon Tae-Young GOO Sueng-Pil JUNG Department of Civil Urban Earth and Environmental Engineering Ulsan National Institute of Science and TechnologyUlsan 44919South Korea Program in Atmospheric and Oceanic Sciences Princeton UniversityPrincetonNew Jersey 08544USA National Institute of Meteorological Sciences NIMSJeju 63568South Korea

This study evaluated the simulation performance of mesoscale convective system(MCS)-induced precipitation,focusing on three selected cases that originated from the Yellow Sea and propagated toward the Korean *** evaluation was conducted for the European Centre for Medium-Range Weather Forecasts(ECMWF)and National Centers for Environmental Prediction(NCEP)analysis data,as well as the simulation result using them as initial and lateral boundary conditions for the Weather Research and Forecasting ***,temperature and humidity profiles from 3D dropsonde observations from the National Center for Meteorological Science of the Korea Meteorological Administration served as validation *** showed that the ECMWF analysis consistently had smaller errors compared to the NCEP analysis,which exhibited a cold and dry bias in the lower levels below 850 *** model,in terms of the precipitation simulations,particularly for high-intensity precipitation over the Yellow Sea,demonstrated higher accuracy when applying ECMWF analysis data as the initial *** advantage also positively influenced the simulation of rainfall events on the Korean Peninsula by reasonably inducing convective-favorable thermodynamic features(i.e.,warm and humid lower-level atmosphere)over the Yellow *** conclusion,this study provides specific information about two global analysis datasets and their impacts on MCS-induced heavy rainfall simulation by employing dropsonde observation ***,it suggests the need to enhance the initial field for MCS-induced heavy rainfall simulation and the applicability of assimilating dropsonde data for this purpose in the future.

关键词： initial conditions dropsonde heavy rainfall forecast global model analysis evaluation

Bone-targeted lipoplex-loaded three-dimensional bioprinting bilayer scaffold enhanced bone regeneration

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Regenerative Biomaterials 2024年第4期3卷 138-148页

作者： Woo-Jin Kim Jeong-hyun Ryu Ji Won Kim Ki-Tae Kim Hye-Rim Shin Heein yoon hyun-Mo Ryoo Young-Dan Cho Department of Molecular Genetics School of Dentistry and Dental Research InstituteSeoul National UniversitySeoul03080Republic of Korea Department of Periodontology School of Dentistry and Dental Research InstituteSeoul National University and Seoul National University Dental HospitalSeoul03080Republic of Korea

Clinical bone-morphogenetic protein 2(BMP2)treatment for bone regeneration,often resulting in complications like soft tissue inflammation and ectopic ossification due to high dosages and non-specific delivery systems,necessitates research into improved biomaterials for better BMP2 stability and *** tackle this challenge,we introduced a groundbreaking bone-targeted,lipoplex-loaded,three-dimensional bioprinted bilayer scaffold,termed the polycaprolactone-bioink-nanoparticle(PBN)scaffold,aimed at boosting bone *** encapsulated BMP2 within the fibroin nanoparticle based lipoplex(Fibroplex)and functionalized it with DSS6 for bone tissue-specific targeting.3D printing technology enables customized,porous PCL scaffolds for bone healing and soft tissue growth,with a two-step bioprinting process creating a cellular lattice structure and a bioink grid using gelatin-alginate hydrogel and DSS6-Fibroplex,shown to support effective nutrient exchange and cell growth at specific pore *** PBN scaffold is predicted through in silico analysis to exhibit biased BMP2 release between bone and soft tissue,a finding validated by in vitro osteogenic differentiation *** PBN scaffold was evaluated for critical calvarial defects,focusing on sustained BMP2 delivery,prevention of soft tissue cell infiltration and controlled fiber membrane pore size in *** PBN scaffold demonstrated a more than eight times longer BMP2 release time than that of the collagen sponge,promoting osteogenic differentiation and bone regeneration in a calvarial defect *** findings suggest that the PBN scaffold enhanced the local concentration of BMP2 in bone defects through sustained release and improved the spatial arrangement of bone formation,thereby reducing the risk of heterotopic ossification.

关键词： 3D bioprinting bone-targeting lipoplex BMP2 bone regeneration

Factor analysis of recent major heatwaves in East Asia

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Geoscience Frontiers 2024年第1期15卷 374-383页

作者： Arim yoon Jeongwon Kim Jooyeop Lee hyun Min Sung Je-Woo Hong Seung-Ki Min Junhong Lee Jinkyu Hong Ecosystem-Atmosphere Process Laboratory Department of Atmospheric SciencesYonsei UniversitySeoul 03722Republic of Korea National Institute of Meteorological Sciences Jeju 63568Republic of Korea Korea Adaptation Center for Climate Korea Environment InstituteSejong 30121Republic of Korea Division of Environmental Science and Engineering Pohang University of Science and TechnologyPohang 37673Republic of Korea Max Planck Institute for Meteorology Hamburg 20146Germany Institute for Convergence Research and Education in Advanced Technology Yonsei UniversitySeoulRepublic of Korea

Heatwaves(HWs)present a major hazard to our society and more extreme heatwaves are expected with future climatic ***,it is important to improve our understanding of the underlying processes that drive HWs,in order to boost our socioeconomic-ecological *** this study,we quantified the influences of key driving factors(large-scale atmospheric circulation,soil moisture,and sea surface temperature)and their synergies on recent heatwaves in East *** conducted a factor separation analysis for three recent HW events by constraining the key factors in the regional Weather Research and Forecasting model with their climatologies or pseudo-observations in different *** study showed distinct spatial variations in the HW-controlling factors in East *** synergistic interaction of large-scale circulation and soil moisture was the most important factors in the 2013 Chinese *** the 2018 HWs in Korea and Japan,the same stagnant large-scale atmospheric circulation played a dominant role in driving the HW *** land-atmosphere coupling via soil moisture,its interaction with circulation,and SST exhibited stronger influences during the Korean HW than the Japanese *** analysis also revealed temporal variations in the factors driving Korean and Chinese HWs due to typhoon passage and other multiple processes over heterogeneous surfaces(i.e.,topographically induced Foehn winds,large-scale warm advection from the warm ocean,spatial differences in soil moisture).Our findings suggest that future heatwave-related studies should consider interactive contributions of key factors,their interplay with surface heterogeneities of complex terrain.

关键词： Heatwave East Asia Large-scale circulation Soil moisture Sea surface temperature Synergistic impacts

Metabolic relationships between marine red algae and algae‑associated bacteria

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Marine Life Science & Technology 2024年第2期6卷 298-314页

作者： Kyung hyun Kim Jeong Min Kim Ju Hye Baek Sang Eun Jeong Hocheol Kim Hwan Su yoon Che Ok Jeon Department of Biological Sciences and Biotechnology Hannam UniversityDaejon 34054Republic of Korea Department of Life Science Chung-Ang UniversitySeoul 06974Republic of Korea Department of Biological Sciences Sungkyunkwan UniversitySuwon 16419Republic of Korea

Mutualistic interactions between marine phototrophs and associated bacteria are an important strategy for their success-ful survival in the ocean,but little is known about their metabolic ***,bacterial communities in the algal sphere(AS)and bulk solution(BS)of nine marine red algal cultures were analyzed,and Roseibium and Phycisphaera were identified significantly more abundantly in AS than in *** metabolic features of Roseibium RMAR6-6(isolated and genome-sequenced),Phycisphaera MAG 12(obtained by metagenomic sequencing),and a marine red alga,Porphyridium purpureum CCMP1328(from GenBank),were analyzed ***6-6 has the genetic capability to fix nitrogen and produce B vitamins(B1,B2,B5,B6,B9,and B12),bacterioferritin,dimethylsulfoniopropionate(DMSP),and phenylacetate that may enhance algal growth,whereas MAG 12 may have a limited metabolic capability,not producing vitamins B9 and B12,DMSP,phenylacetate,and siderophores,but with the ability to produce bacitracin,possibly modulating algal *** CCMP1328 lacks the genetic capability to fix nitrogen and produce vitamin B12,DMSP,phenylacetate,and *** was shown that the nitrogen-fixing ability of RMAR6-6 promoted the growth of ***-pureum,and DMSP reduced the oxidative stress of *** metabolic interactions between strain RMAR6-6 and *** CCMP1328 were also investigated by the transcriptomic analyses of their monoculture and *** together,potential metabolic relationships between Roseibium and *** were *** study provides a bet-ter understanding of the metabolic relationships between marine algae and algae-associated bacteria for successful growth.

关键词： Metabolic relationship Marine red algae Symbiotic bacteria Roseibium Phycisphaera Porphyridium

SARS-CoV-2 aberrantly elevates mitochondrial bioenergetics to induce robust virus propagation

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Signal Transduction and Targeted Therapy 2024年第6期9卷 2702-2715页

作者： Hye Jin Shin Wooseong Lee Keun Bon Ku Gun Young yoon hyun-Woo Moon Chonsaeng Kim Mi-Hwa Kim yoon-Sun Yi Sangmi Jun Bum-Tae Kim Jong-Won Oh Aleem Siddiqui Seong-Jun Kim Department of Convergent Research of Emerging Virus Infection Korea Research Institute of Chemical TechnologyDaejeon 34114Republic of Korea Gyeongnam Biohealth Research Center Gyeongnam Branch InstituteKorea Institute of ToxicologyJinju 52834Republic of Korea Center for Research Equipment Korea Basic Science InstituteCheongjuChungcheongbuk-do 28119Republic of Korea Department of Biotechnology Yonsei UniversitySeoul 03722Republic of Korea Division of Infectious Diseases School of MedicineUniversity of CaliforniaSan DiegoLa JollaCA 92093USA Present address:Department of Microbiology Chungnam National University School of MedicineDaejeon 35015Republic of Korea

Severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)is a‘highly transmissible respiratory pathogen,leading to severe multiorgan ***,knowledge regarding SARS-CoV-2-induced cellular alterations is *** this study,we report that SARSCoV-2 aberrantly elevates mitochondrial bioenergetics and activates the EGFR-mediated cell survival signal cascade during the early stage of viral ***-CoV-2 causes an increase in mitochondrial transmembrane potential via the SARS-CoV-2 RNAnucleocapsid cluster,thereby abnormally promoting mitochondrial elongation and the OXPHOS process,followed by enhancing ATP ***,SARS-CoV-2 activates the EGFR signal cascade and subsequently induces mitochondrial EGFR trafficking,contributing to abnormal OXPHOS process and viral *** EGFR inhibitors remarkably reduce SARS-CoV-2 propagation,among which vandetanib exhibits the highest antiviral *** of SARS-CoV-2-infected cells with vandetanib decreases SARS-CoV-2-induced EGFR trafficking to the mitochondria and restores SARS-CoV-2-induced aberrant elevation in OXPHOS process and ATP generation,thereby resulting in the reduction of SARS-CoV-2 ***,oral administration of vandetanib to SARS-CoV-2-infected hACE2 transgenic mice reduces SARS-CoV-2 propagation in lung tissue and mitigates SARS-CoV-2-induced lung *** also exhibits potent antiviral activity against various SARS-CoV-2 variants of concern,including alpha,beta,delta and omicron,in in vitro cell culture *** together,our findings provide novel insight into SARS-CoV-2-induced alterations in mitochondrial dynamics and EGFR trafficking during the early stage of viral infection and their roles in robust SARS-CoV-2 propagation,suggesting that EGFR is an attractive host target for combating COVID-19.

关键词： alterations aberrant thereby

New anti-mesenteric delta-shaped stapled anastomosis: Technical report with short-term postoperative outcomes in patients with Crohn’s disease

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World Journal of Gastrointestinal Surgery 2024年第8期16卷 2592-2601页

作者： Jong Lyul Lee Yong Sik yoon hyun Gu Lee Young Il Kim Min hyun Kim Chan Wook Kim In Ja Park Seok-Byung Lim Chang Sik Yu Division of Colon and Rectal Surgery Department of SurgeryUniversity of Ulsan College of MedicineAsan Medical CenterSeoul 05505South Korea Department of Surgery Gangdong Kyung Hee University HospitalSeoul 05278South Korea

BACKGROUND Medical treatment for Crohn’s disease(CD)has continuously improved,which has led to a decrease in surgical recurrence *** these advancements,25%of patients will undergo repeat intestinal *** of CD com-monly occurs on the mesentery side of the anastomosis *** To compare the new anti-mesenteric side-to-side delta-shaped stapled anasto-mosis(DSA)with the conventional stapled functional end-to-end anastomosis(CSA).METHODS This retrospective study included CD patients who underwent ileo-ileal or ileo-colic anastomosis between January 2020 and December *** DSA technique employed a stapler to maintain the concept of anti-mesentery side-to-side ana-stomosis by performing a 90°vertical closure of the open window compared with the CSA *** the corner where the open window is closed,the DSA avoids forming a pouch and creates an anastomosis resembling a delta shape within the intestinal *** compared demographics,preoperative condition,operative findings,and operative outcomes for the two *** The study included 175 patients,including 92 in the DSA group and 83 in the CSA *** two groups were similar in baseline characteristics,preoperative medical treatment,and operative findings except for the Montreal classification *** 30-days postoperative complication rate was signi-ficantly lower in the DSA group compared with the CSA group(16.3%vs 32.5%,P=0.009).Ileus incidence was significantly lower in the DSA group than in the CSA group(4.3%vs 14.5%,P=0.033),and the hospital stay was shorter in the DSA group than in the CSA group(5.67±1.53 days vs 7.39±3.68 days,P=0.001).CONCLUSION The DSA technique was feasible and showed comparable postoperative outcomes with lower short-term complic-ations compared with the CSA *** studies on CD recurrence and long-term complications are warranted.

关键词： Crohn’s disease Surgery Anastomosis Complication Recurrence

Galectin 3-binding protein(LGALS3BP)depletion attenuates hepatic fibrosis by reducing transforming growth factor-β1(TGF-β1)availability and inhibits hepatocarcinogenesis

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Cancer Communications 2024年第10期44卷 1106-1129页

作者： Dae-Hwan Kim Minjeong Sung Myong-Suk Park Eun-Gene Sun Sumin yoon Kyung hyun Yoo Kamalakannan Radhakrishnan Sung Yun Jung Woo-Kyun Bae Sang-Hee Cho Ik-Joo Chung Department of Internal Medicine Division of Hematology and OncologyChonnam National University Medical SchoolHwasunSouth Korea Department of Internal Medicine Division of Hematology and OncologyChonnam National University Hwasun HospitalHwasunSouth Korea Combinatorial Tumor Immunotherapy MRC Center Chonnam National University Medical SchoolHwasunSouth Korea National Immunotherapy Innovation Center HwasunSouth Korea BioMedical Sciences Graduate Program Chonnam National UniversityHwasunSouth Korea Department of Biological Science SookmyungWomen’s UniversitySeoulSouth Korea Clinical Vaccine R&D Center Chonnam National UniversityHwasunSouth Korea Department of Biochemistry and Molecular Pharmacology Baylor College of MedicineHoustonTexasUSA

Background:Increased Galectin 3-binding protein(LGALS3BP)serum levels have been used to assess hepatic fibrosis stages and the severity of hepatocellular carcinoma(HCC).Considering the crucial role of transforming growth factor-β1(TGF-β1)in the emergence of these diseases,the present study tested the hypothesis that LGALS3BP regulates the TGF-β1 signaling ***:The expression levels of LGALS3BP and TGFB1 were analyzed in patients with metabolic dysfunction-associated steatohepatitis(MASH)and *** omics techniques,such as RNA-sequencing,transposaseaccessible chromatin-sequencing assay,and liquid chromatography-tandem mass spectrometry proteomics,were used to identify the regulatory mechanisms for the LGALS3BP-TGF-β1 *** effects of altered TGF-β1 signaling by LGALS3BP were investigated in conditional LGALS3BP-knockin and LGALS3BP-knockout ***:In patients with MASH and HCC,the levels of LGALS3BP and TGFB1 exhibited positive *** of LGALS3BP by the inflammatory cytokine interferonαin HCC cells or ectopic overexpression of LGALS3BP in hepatocytes promoted the expression levels of *** fibrosis was observed in the livers of hepatocyte-specific LGALS3BP-knockin mice,with increased TGFB1 ***3BP directly bound to and assembled integrinαV,an integral mediator required for releasing active TGF-β1 from extracellular latent complex with the rearranged F-actin *** released TGF-β1 activated JunB transcription factor,which in turn promoted the TGF-β1 positive feedback ***3BP deletion in the hepatocytes downregulated TGF-β1 signaling and CCl4 induced ***,LGALS3BP depletion hindered hepatocarcinogenesis by limiting the availability of fibrogenic TGF-β***:LGALS3BP plays a crucial role in hepatic fibrosis and carcinogenesis by controlling the TGF-β1 signaling pathway,making it a promising therapeutic target in TGF-β1-related diseases.

关键词： F-actin FAK hepatic carcinogenesis IntegrinαV Interferonα JunB LGALS3BP metabolic dysfuntion-associated steatohepatitis tensile force TGF-β1