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Recent development and gene therapy for glycogen storage disease type Ia

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Liver Research 2017年第3期1卷 174-180页

作者： Janice Y.Chou Goo-Young kim Jun-Ho Cho Section on Cellular Differentiation Eunice Kennedy Shriver National Institute of Child Health and Human DevelopmentNational Institutes of HealthBethesdaMDUSA

Glycogen storage disease type Ia(GSD-Ia)is an autosomal recessive metabolic disorder caused by a deficiency in glucose-6-phosphatase-α(G6Pase-αor G6PC)that is expressed primarily in the liver,kidney,and intestine.G6Pase-αcatalyzes the hydrolysis of glucose-6-phosphate(G6P)to glucose and phosphate in the terminal step of gluconeogenesis and glycogenolysis,and is a key enzyme for endogenous glucose *** active site of G6Pase-αis inside the endoplasmic reticulum(ER)*** catalysis,the substrate G6P must be translocated from the cytoplasm into the ER lumen by a G6P transporter(G6PT).The functional coupling of G6Pase-αand G6PT maintains interprandial glucose *** therapies for GSD-Ia are available,but cannot prevent the long-term complication of hepatocellular adenoma that may undergo malignant transformation to hepatocellular *** models of GSD-Ia are now available and are being exploited to both delineate the disease more precisely and develop new treatment approaches,including gene therapy.

关键词： Glycogen storage disease type Ia(GSD-Ia) Glucose-6-phosphatase-α(G6Pase-αor G6PC) Gene therapy Hepatocellular adenoma Recombinant adeno-associated virus vector

Do brood parasitic Common Cuckoos develop brood patches during the breeding season?

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Avian Research 2024年第3期15卷 363-368页

作者： Jun-Seo Go Hae-Ni kim Sue-Jeong Jin Myeong-Chan Cha Heesoo Lee Jin-Won Lee Korea Institute of Ornithology&Department of Biology Kyung Hee University02447SeoulRepublic of Korea Bird Research SaeZiP 12504YangpyeongRepublic of Korea

Many birds develop brood patches on their ventral apterium under hormonal regulation to effectively transmit body heat to eggs during *** developed patch has several characteristics,including de-feathering,vascularization,and ***,little is known about whether avian brood parasites that do not incubate their eggs exhibit brood patch development during the breeding *** this study,we measured the size of the ventral apterium in 114 Common Cuckoos(Cuculus canorus)captured in the field throughout the breeding season and examined the appearance of the ventral apterium to confirm the development of brood *** also examined whether morphological traits and various factors correlated with the size of the ventral apterium(sternal apterium and abdominal apterium)and how it changed during the breeding *** found no clear signs of brood patch development in Common Cuckoos captured throughout the breeding season,indicating that they likely did not develop brood patches on the ventral apterium during this *** also found that ventral apterium size was positively correlated with wing length and body *** addition,Common Cuckoos with newly growing feathers on the boundary of the ventral apterium with pteryla were frequently observed as seasons progressed to the *** conclusion,Common Cuckoos exhibit neither brood patch development nor the vestigial characteristics of ancestral brood patches,except for the growth of feathers on boundary the ventral *** studies examining brood patches across different groups of avian brood parasites are valuable for enhancing our understanding of the developmental and physiological adaptations of avian brood parasites.

关键词： Brood parasitism Brood patch Common Cuckoo Pterylosis Re-feathering Ventral apterium

Innovative confocal laser method for exact dioptric power measurement of intraocular lens implants Invited Paper

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Chinese Optics Letters 2008年第12期6卷 876-878页

作者： Ilko K.Ilev Robert W.Faaland Do-Hyun kim Robert H.James Don Calogero Office of Science and Engineering Laboratories Center for Devices and Radiological Health U.S.Food and Drug Administration10903 New Hampshire AvenueSilver Spring Office of Device Evaluation Center for Devices and Radiological Health U.S.Food and Drug Administration9200 Corporate BoulevardRockville

We present a novel confocal laser method （CLM） for precise testing of the dioptric power of both positive and negative intraocular lens （IOL） implants. The CLM principle is based on a simple fiber-optic confocal laser design including a single-mode fiber coupler that serves simultaneously as a point light source used for formation of a collimated Gaussian laser beam, and as a highly sensitive confocal point receiver. The CLM approach provides an accurate, repeatable, objective, and fast method for IOL dioptric power measurement over the range from 0 D to greater than ±30 D under both dry and in-situ simulated conditions.

关键词： Crystalline lens Fiber optic couplers Gaussian beams Intraocular lenses Laser beams Single mode fibers

Electric field enhancement of coupled plasmonic nanostructures for optical amplification

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PhotoniX 2023年第1期4卷 579-591页

作者： Jun Hyun kim Ja Yeon Lee Eung Soo kim Myung Yung Jeong Department of Cogno-mechatronics Engineering Pusan National UniversityBusan 46241Korea ESPn Medic ESPn Medic cooperationBusan 46241Korea Department of Electronic&Robotics Engineering Busan University of Foreign StudiesBusan 46234Korea Department of Optics and Mechatronics Engineering Pusan National UniversityBusan 46241Korea

Plasmonic effects that enhance electric fields and amplify optical signals are crucial for improving the resolution of optical imaging systems. In this paper, a metal-based plasmonic nanostructure (MPN) is designed to increase the resolution of an optical imaging system by amplifying a specific signal while producing a plasmonic effect via a dipole nanoantenna (DN) and grating nanostructure (GN), which couple the electric field to be focused at the center of the unit cell. We confirmed that the MPN enhances electric fields 15 times more than the DN and GN, enabling the acquisition of finely resolved optical signals. The experiments confirmed that compared with the initial laser intensity, the MPN, which was fabricated by nanoimprint lithography, enhanced the optical signal of the laser by 2.24 times. Moreover, when the MPN was applied in two optical imaging systems, an indistinguishable signal that was similar to noise in original was distinguished by amplifying the optical signal as 106 times in functional near-infrared spectroscopy(fNIRS), and a specific wavelength was enhanced in fluorescence image. Thus, the incorporation of this nanostructure increased the utility of the collected data and could enhance optical signals in optics, bioimaging, and biology applications.

关键词： Plasmonic effect Nanostructure Nanoimprint lithography Field enhancement Amplifying optical intensity Selective deposition fabrication

Interactions between Transmembrane Helices within Monomers of the Aquaporin AtPIP2;1 Play a Crucial Role in Tetramer Formation

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Molecular Plant 2016年第7期9卷 1004-1017页

作者： Yun-Joo Yoo Hyun Kyung Lee Wonhee Han Dae Heon kim Myoung Hui Lee Jouhyun Jeon Dong Wook Lee Junho Lee Yongjik Lee Juhun Lee Jin Seok kim Yunje Cho Jin-Kwan Han Inhwan Hwang School of Interdisciplinary Bioscience and Bioengineering Pohang University of Science and Technology Pohang 790-784 Korea Department of Life Sciences Pohang University of Science and Technology Pohang 790-784 Korea Department of Biology Sunchon National University Sunchon 57922 Korea Division of Integrative Biosciences and Biotechnology Pohang University of Science and Technology Pohang 790-784 Korea

Aquaporin （AQP） is a water channel protein found in various subcellular membranes of both prokaryotic and eukaryotic cells. The physiological functions of AQPs have been elucidated in many organisms. However, understanding their biogenesis remains elusive, particularly regarding how they assemble into tetramers. Here, we investigated the amino acid residues involved in the tetramer formation of the Arabidopsis plasma membrane AQP AtPIP2;1 using extensive amino acid substitution mutagenesis. The mutant proteins V41A/ E44A, F51A/L52A, F87A/191A, F92A/193A, V95A/Y96A, and H216A/L217A, harboring alanine substitutions in the transmembrane （TM） helices of AtPIP2;1 polymerized into multiple oligomeric complexes with a vari- able number of subunits greater than four. Moreover, these mutant proteins failed to traffic to the plasma membrane, instead of accumulating in the endoplasmic reticulum （ER）. Structure-based modeling revealed that these residues are largely involved in interactions between TM helices within monomers. These results suggest that inter-TM interactions occurring both within and between monomers play crucial roles in tetramer formation in the AtPIP2;1 complex. Moreover, the assembly of AtPIP2;1 tetramers is critical for their trafficking from the ER to the plasma membrane, as well as water permeability.

关键词： aquaporin AtPIP2 tetramer formation interaction between transmembrane helices

Autonomous self-healing 3D micro-suction adhesives for multi-layered amphibious soft skin electronics

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InfoMat 2024年第10期6卷 65-79页

作者： Dohyun Lim Min Woo Jeong Hyeongho Min Yeon Soo Lee Gui Won Hwang Seung Hwan Jeon Kyu Ho Jung Ngoc Thanh Phuong Vo Min-Seok kim Da Wan kim Jin Young Oh Changhyun Pang School of Chemical Engineering Sungkyunkwan University(SKKU)SuwonGyeonggi-doRepublic of Korea Department of Chemical Engineering(Integrated Engineering Program) Kyung Hee UniversityYonginGyeonggiRepublic of Korea Convergence Research Group for Meta-Touch Korea Research Institute of Standards and ScienceDaejeonRepublic of Korea Department of Electronic Engineering Korea National University of TransportationChungju-siChungbukRepublic of Korea Samsung Advanced Institute for Health Sciences and Technology(SAIHST) Sungkyunkwan UniversitySuwonGyunggi-doRepublic of Korea

Autonomously self-healing, reversible, and soft adhesive microarchitecturesand structured electric elements could be important features in stable and versatilebioelectronic devices adhere to complex surfaces of the human body(rough, dry, wet, and vulnerable). In this study, we propose an autonomousself-healing multi-layered adhesive patch inspired by the octopus, which possessself-healing and robust adhesion properties in dry/underwater *** implement autonomously self-healing octopus-inspired architectures, adynamic polymer reflow model based on structural and material design suggestscriteria for three-dimensional patterning self-healing elastomers. In addition,self-healing multi-layered microstructures with different moduli endowsefficient self-healing ability, human-friendly reversible bio-adhesion, and stablemechanical deformability. Through programmed molecular behavior ofmicrolevel hybrid multiscale architectures, the bioinspired adhesive patchexhibited robust adhesion against rough skin surface under both dry andunderwater conditions while enabling autonomous adhesion restoring performanceafter damaged (over 95% healing efficiency under both conditions for24 h at 30℃). Finally, we developed a self-healing skin-mountable adhesiveelectronics with repeated attachment and minimal skin irritation by laminatingthin gold electrodes on octopus-like structures. Based on the robust adhesionand intimate contact with skin, we successfully obtained reliable measurements during dynamic motion under dry, wet, and damagedconditions.

关键词： biomimetics dry adhesive self-healing polymer stretchable electronics

A Fully CMOS Integrated RF Transceiver for Ubiquitous Sensor Networks in Sub-GHz ISM-band

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A Fully CMOS Integrated RF Transceiver for Ubiquitous Sensor...

Foaming supramolecular surfactants for gas mobility control in naturally fractured carbonate reservoirs at high temperature,salinity,and hardness

2005 6th International Conference on ASIC

作者： Hae-Moon Seo Yeon-Kuk Moon Yong-Kuk Park Kwang-Ho Won Myung-Hyun Yoon Jun-jae Yoo Sung-Dong kim Ubiquitous Computing Research Center Korea Electronics Technology Institute（KETI） 68Yatap-DongBundang-GuSungnam-SiKyungGi-DoKorea

＜正＞A fully CMOS integrated radio frequency （RF） transceiver for wireless sensor networks in sub-GHz ISM-band applications is implemented and measured. The IC is fabricated in 0.18- m CMOS technology and packaged in LPCC *** fully monolithic transceiver consists of a receiver,a transmitter and a RF synthesizer with on-chip *** chip fully complies with the IEEE 802.15.4 WPAN standard in sub-GHz *** receiver sensitivity is 98dBm and the transmitter achieves less than 6.3 error vector magnitude（EVM） for 40kbps *** chip uses 1.8V power supply and the current consumption is 14mA for reception mode ad 16mA for transmission mode.

关键词： nde terms wireless sensor network integrated circuit CMOS RF transceiver low power

来源： cnki会议评论

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Petroleum Science 2022年第6期19卷 3134-3148页

作者： Enrique Soto-Castruita Raúl Hern andez-Altamirano Eduardo Buenrostro-González Erick-Emanuel Luna-Rojero Sung jae Ko-kim Violeta-Yasmín Mena-Cervantes Mirna Pons-Jiménez Jorge-Francisco Ramírez-Pérez David-Aaσn Nieto-Alvarez Ricardo Cerón-Camacho Jos e-Ernesto Parra Raúl Oviedo-Roa Jos e-Manuel Martínez-Magadán Rodolfo Cisneros-Dévora Luis-S.Zamudio-Rivera Instituto Mexicano del Petr oleo Eje Central Lazaro Cardenas Norte 152Col.San Bartolo AtepehuacanCiudad de Mexico07730Mexico CONACy T-Instituto Mexicano del Petroleo Eje Central Lazaro Cardenas Norte 152Col.San Bartolo AtepehuacanCiudad de Mexico07730Mexico Centro Mexicano para la Produccion mas Limpia Instituto Politecnico NacionalAvenida Acueducto s/nCol.La Laguna TicomanCiudad de Mexico07340Mexico Centro de Tecnologías para Exploracion y Produccion Camino de Terracería 800Col.San Jose NovilleroBoca delRíoVeracruz94286Mexico

Oil production and mainte nance are essential issues in naturally fractured reservoirs because they are the largest and most productive on ***,they present early water and gas channeling but could be remediated by using foaming agents to control these phenomena through blocking channeling *** Mexico these reservoirs have pressure up to 5,500 psi,high temperature up to 200℃,salinity up to400,000 ppm,and hardness up to 250,000 ppm;due to these thermodynamic conditions,there has been no available technology to form stable enough *** this work,a foaming supramolecular surfactant with the capability to chelate Ca^(2+)ions is *** a result,surfactant monomers are bridged by captured Ca^(2+)cations leading to the formation of high-molecular-weight oligomers,which significantly increment the viscosity of the solution improving the foam stability,and since at this manner the Ca2+cations are no longer available to precipitate as components of solid salts,the foaming supramolecular surfactant also performs as *** observations are explained through quantum theoretical *** foam is stable,effectively blocking the gas channels,whereas in presence of oil the foam is broken leading the oil to pass into the *** characteristic rheological properties of the foam allow its injection into the formation at a range of flow rates,foam qualities,and shear stress to achieve the flooding and the blocking of a variety of fractured carbonate formations,and the change of the wettability of the matrix,which is a desirable behavior in a huff and puff process,as reported in a previous publication about a successful pilot test of this foam.

关键词： Calcium chelation Foaming composition Supramolecular surfactant Molecular design Gas mobility control Flow assurance Improved oil recovery Conformance control

4-Hydroxycinnamic acid attenuates neuronal cell death by inducing expression of plasma membrane redox enzymes and improving mitochondrial functions

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Food Science and Human Wellness 2023年第4期12卷 1287-1299页

作者： Sujin Park YoonA kim jaewang Lee Hyunsoo Seo Sang-Jip Nam Dong-Gyu Jo Dong-Hoon Hyun Department of Life Science Ewha Womans UniversitySeoul 03760South Korea Department of Chemistry and Nano Science Ewha Womans UniversitySeoul 03760South Korea School of Pharmacy Sungkyunkwan UniversitySuwon 16419South Korea

Many approaches to neurodegenerative diseases that focus on amyloid-βclearance and gene therapy have not been *** therapeutic applications focus on enhancing neuronal cell survival during the pathogenesis of neurodegenerative diseases,including mitochondrial *** membrane(PM)redox enzymes are crucial in maintaining cellular physiology and redox homeostasis in response to mitochondrial *** phytochemicals are known to induce the expression of detoxifying enzymes under stress *** this study,mechanisms of neuroprotective effects of 4-hydroxycinnamic acid(HCA)were examined by analyzing cell survival,levels of abnormal proteins,and mitochondrial functions in two different neuronal *** protected two neuronal cells exhibited high expression of PM redox enzymes and the consequent increase in the NAD^(+)/NADH *** cultured with HCA showed delayed apoptosis and decreased oxidative/nitrative damage accompanied by decreased ROS production in the *** increased the mitochondrial complexes I and II activities and ATP ***,HCA increased mitochondrial fusion and decreased mitochondrial ***,HCA maintains redox homeostasis and energy metabolism under oxidative/metabolic stress *** findings suggest that HCA could be a promising therapeutic approach for neurodegenerative diseases.

关键词： NADH-quinone oxidoreductase 1(NQO1) Cytochrome b5 reductase 4-Hydroxycinnamic acid Neuroprotection Improved mitochondrial functions