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Emerging concepts underlying selective neuromuscular dysfunction in infantile-onset spinal muscular atrophy

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Neural Regeneration Research 2021年第10期16卷 1978-1984页

作者： kishore Gollapalli jeong-ki kim Umrao R.Monani Department of Neurology Columbia University Medical CenterNew YorkNYUSA Department of Pathology&Cell Biology Columbia University Medical CenterNew YorkNYUSA Center for Motor Neuron Biology and Disease Columbia University Medical CenterNew YorkNYUSA

Infantile-onset spinal muscular atrophy is the quintessential example of a disorder characterized by a predominantly neurodegenerative phenotype that nevertheless stems from perturbations in a housekeeping *** from low levels of the Survival of Motor Neuron(SMN)protein,spinal muscular atrophy manifests mainly as a lower motor neuron *** this is so and whether other cell types contribute to the classic spinal muscular atrophy phenotype continue to be the subject of intense investigation and are only now gaining ***,what is emerging is sometimes as puzzling as it is instructive,arguing for a careful re-examination of recent study outcomes,raising questions about established dogma in the field and making the case for a greater focus on milder spinal muscular atrophy models as tools to identify key mechanisms driving selective neuromuscular dysfunction in the *** review examines the evidence for novel molecular and cellular mechanisms that have recently been implicated in spinal muscular atrophy,highlights breakthroughs,points out caveats and poses questions that ought to serve as the basis of new investigations to better understand and treat this and other more common neurodegenerative disorders.

关键词： motor neuron neurodegeneration neuromuscular spinal muscular atrophy splicing

Naturally occurring mutations in the reverse transcriptase region of hepatitis B virus polymerase from treatment-na?ve Korean patients infected with genotype C2

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World Journal of Gastroenterology 2017年第23期23卷 4222-4232页

作者： Ji-Eun kim So-Young Lee Hong kim ki-jeong kim Won-Hyeok Choe Bum-Joon kim Department of Biomedical Sciences Microbiology and Immunologyand Liver Research InstituteSeoul National University College of Medicine Department of Microbiology School of MedicineJoong-Ang University Department of Internal Medicine Konkuk University School of Medicine

AIM To report naturally occurring mutations in the reverse transcriptase region(RT) of hepatitis B virus(HBV) polymerase from treatment na?ve Korean chronic patients infected with genotype *** Here, full-length HBV reverse transcriptase RT sequences were amplified and sequenced from 131 treatment na?ve Korean patients chronically infected with hepatitis B genotype C2. The patients had two distinct clinical statuses: 59 patients with chronic hepatitis(CH) and 72 patients with hepatocellular carcinoma(HCC). The deduced amino acids(AAs) at42 previously reported potential nucleos(t)ide analog resistance(NAr) mutation positions in the RT region were analyzed. RESULTS Potential NAr mutations involving 24 positions were found in 79 of the 131 patients(60.3%). Notably, AA substitutions at 2 positions(rt184 and rt204) involved in primary drug resistance and at 2 positions(rt80 and rt180) that functioned as secondary/compensatory mutations were detected in 10 patients(1 CH patient and 9 HCC patients) and 7 patients(1 CH and 6 HCC patients), respectively. The overall mutation frequencies in the HCC patients(3.17%, 96/3024 mutations) were significantly higher than the frequencies in the CH patients(2.09%, 52/2478 mutations)(P = 0.003). In addition, a total of 3 NAr positions, rt80, rt139 and rt204 were found to be significantly related to HCC from treatment na?ve Korean patients. CONCLUSION Our data showed that naturally occurring NAr mutations in South Korea might contribute to liver disease progression(particularly HCC generation) in chronic patients with genotype C2 infections.

关键词： Hepatitis B virus Polymerase Reverse transcriptase Potential nucleos(t)ide analog resistance Chronic hepatitis Hepatocellular carcinoma

Hepatitis B virus pre S1 deletion is related to viral replication increase and disease progression

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World Journal of Gastroenterology 2015年第16期21卷 5039-5048页

作者： Seoung-Ae Lee ki-jeong kim Hong kim Won-Hyuk Choi Yu-Sub Won Bum-Joon kim Department of Biomedical Sciences Microbiology and Immunologyand Liver Research InstituteSeoul National University College of Medicine Department of Microbiology School of MedicineChung-Ang University Department of Internal Medicine Konkuk University

AIM:To investigate the clinical implications of hepatitis B virus(HBV) pre S1 ***:We developed a fluorescence resonance energy transfer-based real-time polymerase chain reaction(RT-PCR) that can detect four genotypes(wild type, 15-bp, 18-bp and 21-bp deletion).The PCR method was used in two cohorts of Korean chronic HBV subjects with genotype C *** Ⅰ included 292 chronic HBV subjects randomly selected from Cheju National University Hospital(Jeju, South Korea) or Seoul National University Hospital(Seoul, South Korea), and cohort Ⅱ included 90 consecutive chronic HBV carriers recruited from Konkuk University Hospital(Seoul, South Korea); the cohort Ⅱ patients did not have hepatocellular carcinoma or liver ***:The method proposed in this study identified 341 of 382 samples(89.3%).Deletion variants were identified in 100(29.3%) of the 341 detected *** both cohorts, the subjects with deletions had a significantly higher Hepatitis B virus e antigen(HBe Ag)-positive seroprevalence [cohort Ⅰ, wild(51.0%) vs deletion(75.0%), P < 0.001; cohort Ⅱ, wild(69.2%) vs deletion(92.9%), P = 0.002] and higher HBV DNA levels [cohort Ⅰ, wild(797.7 pg/m L) vs deletion(1678.9 pg/m L), P = 0.013; cohort Ⅱ, wild(8.3 × 108 copies/m L) vs deletion(2.2 × 109 copies/m L), P = 0.049], compared to subjects with wild type ***:HBV genotype C pre S1 deletion may affect disease progression in chronic HBV subjects through an extended duration of HBe Ag seropositive status and increased HBV replications.

关键词： Hepatitis B virus PreS1 start codon deletion Hepatitis B virus e antigen Hepatocellular carcinoma Genotype C

Effects of humic acid on phytodegradation of petroleum hydrocarbons in soil simultaneously contaminated with heavy metals

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Journal of Environmental Sciences 2011年第12期23卷 2034-2041页

作者： Soyoung Park ki Seob kim jeong-Tae kim Daeseok Kang kijune Sung Department of Ocean Engineering Pukyong National University Busan 608-737 Korea Department of Ecological Engineering Pukyong National University Busan 608-737 Korea

The use of humic acid （HA） to enhance the efficiency of phytodegradation of petroleum hydrocarbons in soil contaminated with diesel fuel was evaluated in this study. A sample of soil was artificially contaminated with commercially available diesel fuel to an initial total petroleum hydrocarbons （TPH） concentration of 2300 mg/kg and four heavy metals with concentrations of 400 mg/kg for Pb, 200 mg/kg for Cu, 12 mg/kg for Cd, and 160 mg/kg for Ni. Three plant species, Brassica campestris, Festuca arundinacea, and Helianthus annuus, were selected for the phytodegradation experiment. Percentage degradation of TPH in the soil in a control pot supplemented with HA increased to 45% from 30% without HA. The addition of HA resulted in an increases in the removal of TPH from the soil in pots planted with B. campestris, F. arundinacea, and H. annuus, enhancing percentage degradation to 86%, 64%, and 85% from 45%, 54%, and 66%, respectively. The effect of HA was also observed in the degradation of n-alkanes within 30 days. The rates of removal of n-alkanes in soil planted with B. campestris and H. annuus were high for n-alkanes in the range of C11–C28. A dynamic increase in dehydrogenase activity was observed during the last 15 days of a 30-day experimental period in all the pots amended with HA. The enhanced biodegradation performance for TPHs observed might be due to an increase in microbial activities and bioavailable TPH in soils caused by combined effects of plants and HA. The results suggested that HA could act as an enhancing agent for phytodegradation of petroleum hydrocarbons in soil contaminated with diesel fuel and heavy metals.

关键词： phytodegradation TPHs humic acid n-alkanes DHA combined effects

Mechanical and electrochemical characteristics evaluation in annealing treatment for ship material

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中国有色金属学会会刊：英文版 2009年第B09期19卷 40-44页

作者： Seok-ki JANG Young-Jae jeong Seong-Jong kim Division of Marine Engineering Mokpo Maritime University Jeonnam Techno Park

The effect of heat treatment on the electrochemical and mechanical properties of SS400 steel was investigated, and the effects of annealing conditions on notched specimens subjected to the slow strain rate test (SSRT) were studied. The results show that no correlations are observed among the maximum tensile strength, yield strength, and stress corrosion cracking. In contrast, the elongation and time-to-fracture are improved with stress-relieving annealing compared with the as-received condition. The elongation, time-to-fracture, and number of dimples increase with annealing heat treatment.

关键词：退火处理电化学船舶材料特性评价机械 SS400钢应力腐蚀开裂消除应力退火

New para-magnetic(CoFeNi)(50)(CrMo)(50-x)(CB)x(x=20,25,30)non-equiatomic high entropy metallic glasses with wide supercooled liquid region and excellent mechanical properties

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Journal of Materials Science & Technology 2020年第8期36卷 135-143页

作者： jeong Tae kim Sung Hwan Hong Jin Man Park Jürgen Eckert ki Buem kim Erich Schmid Institute of Materials Science Austrian Academy of Sciences(ÖAW)Jahnstrae 128700LeobenAustria Hybrid Materials Center(HMC) Department of Nanotechnology and Advanced Materials EngineeringSejong University209Neungdong-roGwangjin-guSeoul143-747Republic of Korea Global Technology Center(GTC) Samsung Electronics Co.Ltd129 Samsung-roYeongtong-guSuwonsiGyeonggi-do443-742Republic of Korea Department of Materials Science Chair of Materials PhysicsMontanuniversität LeobenJahnstraße 128700LeobenAustria

In this study,high entropy metallic glasses(HEMGs)were developed through a combination of concepts for designing metallic glasses(main element+transition metal+metalloid element)and high-entropy alloys(more than five elements,each element having an atomic concentration between 5 at.%and 35 at.%).The developed metallic glass alloys are composed of Co-Fe-Ni main elements,transition metals(Cr,Mo)and metalloid elements(C,B).Moreover,the present work reports the thermal,mechanical and magnetic properties of(CoFeNi)(50)(CrMo)(50-x)(CB)x alloys with x=20,25,*** developed as-spun HEMGs exhibit typical paramagnetic properties even for a high amount of ferromagnetic elements(Co,Fe,and Ni)and have high elastic modulus(103–160 GPa)and hardness(14–27 GPa),thus possessing mechanical properties similar to well-known Co-based metallic glasses(Co-Cr-Mo-C-B system).In addition,some of the bulk samples prepared with a diameter of 2 mm form bulk metallic glasses with a high compressive strength around 3.5 *** mechanisms determining the stability of the supercooled liquid,as well as the paramagnetic and mechanical properties for the developed non-equiatomic HEMGs,are discussed.

关键词： Metallic glasses Paramagnetism High entropy alloys Phase stability

A Case Study of Mesoscale Convective Band(MCB) Development and Evolution along a Quasi-stationary Front

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Advances in Atmospheric Sciences 2014年第4期31卷 901-915页

作者： Daeun jeong ki-Hong MIN Gyuwon LEE Kyung-Eak kim Climate Research Department Asia-Pacific Economic Cooperation Climate Center Center for Atmospheric Remote Sensing Kyungpook National University Department of Astronomy and Atmospheric Sciences Kyungpook National University Department of Earth Atmospheric and Planetary Sciences Purdue University

This paper presents a case study of mesoscale convective band （MCB） development along a quasi-stationary front over the Seout metropolitan *** MCB,which initiated on 1500 UTC 20 September 2010 and ended on 1400 UTC 21 September 2010,produced a total precipitation amount of 259.5 *** MCB development occurred during a period of tropopause folding in the upper level and moisture advection with a low-level *** analyses show that the evolution of the MCB can be classified into five periods：（1） the cell-forming period,when convection initiated; （2） the frontogenetic period,when the stationary front formed over the Korean peninsula; （3） the quasi-stationary period,when the convective band remained over Seoul for 3 h; （4） the mature period,when the cloud cover was largest and the precipitation rate was greater than 90 mm h-1; and （5） the dissipating period,when the MCB diminished and *** synoptic,thermodynamic,and dynamic analyses show that the MCB maintained its longevity by a tilted updraft,which headed towards a positive PV *** was concentrated under this area,where a tilted ascending southwesterly converged with a tilted ascending northeasterly,at the axis of cyclonic *** formation of the convective cell was attributed in part by tropopause folding,which enhanced the cyclonic vorticity at the surface,and by the low-level convergence of warm moist air and upperlevel *** southwesterly flow ascended in a region with high moisture content and strong relative vorticity that maintained the development of an MCB along the quasi-stationary front.

关键词： convective band quasi-stationary front potential vorticity tropopause folding heavy precipitation

Biologically inspired ultrathin arrayed camera for high-contrast and high-resolution imaging

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Light(Science & Applications) 2020年第1期9卷 1742-1748页

作者： kisoo kim Kyung-Won Jang Jae-Kwan Ryu ki-Hun jeong Department of Bio and Brain Engineering Korea Advanced Institute of Science and Technology(KAIST)291 Daehak-roYuseong-guDaejeon 34141Republic of Korea KAIST Institute for Health Science and Technology KAISTDaejeon 34141Republic of Korea Unmanned/Robotic Systems Lab. LIG Nex1 Co.LtdSeongnam 13488Republic of Korea

Compound eyes found in insects provide intriguing sources of biological inspiration for miniaturised imaging ***,we report an ultrathin arrayed camera inspired by insect eye structures for high-contrast and super-resolution *** ultrathin camera features micro-optical elements(MOEs),i.e.,inverted microlenses,multilayered pinhole arrays,and gap spacers on an image *** MOE was fabricated by using repeated photolithography and thermal *** fully packaged camera shows a total track length of 740μm and a field-of-view(FOV)of 73°.The experimental results demonstrate that the multilayered pinhole of the MOE allows high-contrast imaging by eliminating the optical crosstalk between *** integral image reconstructed from array images clearly increases the modulation transfer function(MTF)by~1.57 times compared to that of a single channel image in the ultrathin *** ultrathin arrayed camera provides a novel and practical direction for diverse mobile,surveillance or medical applications.

关键词： resolution high camera

Advanced heterolytic H_(2) adsorption of K-added Ru/MgO catalysts for accelerating hydrogen storage into aromatic benzyltoluenes

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Journal of Energy Chemistry 2022年第8期31卷 333-343,I0009页

作者： Tae Wan kim Hwiram jeong Yeongin Jo Dongun kim Ji Hoon Park Seok ki kim Young-Woong Suh Department of Chemical Engineering Hanyang UniversitySeoul 04763Republic of Korea Environmental&Sustainable Resources Research Center Korea Research Institute of Chemical TechnologyDaejeon 34114Republic of Korea Department of Chemical Engineering Ajou UniversitySuwon 16499Republic of Korea

Herein,we report a highly active K-added Ru/MgO catalyst for hydrogen storage into aromatic benzyltoluenes at low temperatures to advance liquid organic hydrogen carrier *** hydrogenation activity of Ru/K/MgO catalysts exhibits a volcano-shaped dependence on the K content at the maximum with 0.02 wt%.This is in good agreement with the strength and capacity of H_(2) adsorption derived from basicity,despite a gradual decrease in the textural property and the corresponding increase in the Ru particle size with increasing the K *** functional theory calculations show that heterolytic hydrogen adsorption properties(strength and polarization)are facilitated up to a specific density of K on the Ru–MgO interface and excessive K suppresses heterolytic H_(2) adsorption by direct interaction between K and hydrogen,assuring the hydrogenation activity and H_(2) adsorption capability of Ru/K/MgO ***,the Ru/K/MgO catalyst,when K is added in an optimal amount,is highly effective to accelerate hydrogen storage kinetics at low temperatures owing to the enhanced heterolytic H_(2) adsorption.

关键词： Chemical hydrogen storage Supported Ru catalysts Ru–MgO interface Heterolytic H_(2)adsorption Charge transfer Potassium promotion