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WHIM Syndrome With a Novel CXCR4 Variant in a Korean Child
신동우,박시내,김성민,임경옥,김정아,홍경택,최정윤,홍채리,박경덕,신희영,강형진,김현경,이동순 대한진단검사의학회 2017 Annals of Laboratory Medicine Vol.37 No.5
Dear Editor, Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a rare, autosomal dominant, primary immunodeficiency disorder [1], with only 65 cases reported worldwide [2]. This disorder is caused by a gain-of-function mutation in CXCR4, which, along with its ligand CXCL12 (stromal cell-derived factor 1 [SDF-1]), plays a role in retention of mature neutrophils in the bone marrow (BM) [3]. Here we describe a Korean child with WHIM syndrome who had a novel CXCR4 mutation. To the best of our knowledge, this is the first report of WHIM syndrome in Korea.
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A model of fracture toughness in fibrous Si3N4 monolithic ceramics
신동우,Dae-Hyun Yoona,Hai Guo 한양대학교 세라믹연구소 2005 Journal of Ceramic Processing Research Vol.6 No.4
A fracture toughness model describing the fracture behavior of fibrous monolithic ceramics, based on the analysis of the energy of interfacial cracks is presented. The results reveal that the only factor that contributes to the fracture toughness in ceramics was the actual energy absorbed by crack propagation and not the total work of fracture. The load-displacement curve and the crack propagation path were predicted using a derived model which was the mirror image with that of the experimental data. The influence of unit cell dimension, strength, and interfacial bonding strength on properties of the ceramics were predicted using a fracture toughness model which matched with the experimental results. The deviation in results was less than 10% which indicates the validity of the proposed model.
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Fabrication and mechanical properties of fibrous silicon nitride monolithic ceramics
신동우,Dae-Hyun Yoon,Hai Guo 한양대학교 세라믹연구소 2005 Journal of Ceramic Processing Research Vol.6 No.4
Fibrous Si3N4 monolithic ceramics with a hexagonal cell (Si3N4 fiber) and diameters in the range of 0.1-0.5 mm were processed using a composite fabrication method with a high preferred orientation by the tape casting of seeds of rodlike β-Si3N4 single crystals. The densified Si3N4 fibrous monolith showed a fracture toughness of 17 MPa·m1/2 and a three-point bend strength of 690 MPa. The application of reinforcement with SiC whiskers exhibited a fracture toughness (24 MPa·m1/2) and strength (705 MPa). Grain orientation, interface, and reinforcement played a deciding role in mechanical properties of these monolithic ceramics.
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A fracture model and prediction of mechanical behavior in Si3N4 laminate composites
신동우,Hai Guo 한양대학교 세라믹연구소 2005 Journal of Ceramic Processing Research Vol.6 No.3
A fracture model is proposed to describe the fracture behavior of laminated ceramic composites. The results reveal that the actual energy absorbed, Wact, by crack propagation is more suitable than work-of-fracture to calculate the toughness of composites. Fracture toughness of composites is obtained using the proposed model taking actual energy absorption into account. The predicted load-displacement curve matches with the experimental results. The results based on the proposed model regarding the influence of layer thickness and the interfacial bonding strength on fracture toughness hold valid with the calculated range.
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백서 혈관평활근 세포에서 α-Lipoic acid가 PAI-1 발현, 세포의 증식, 주유능 및 신생내막 형성억제에 미치는 효과
신동우,이동욱,이상준,김혜순,강효경,안종덕,이인규 대한당뇨병학회 2002 Diabetes and Metabolism Journal Vol.25 No.6
연구배경:당뇨병의 혈관 합병증의 발생에 있어서 산화스트레스는 중요한 역할을 하는 것으로 알려져 있다. 혈관 평활근세포에서 고혈당은 안지오텐신Ⅱ와 더불어 활성산소족(ROS)을 증가시키며, 산화스트레스에 민감한 전사인자들을 활성화시켜 동맥경화증을 유발하는 것으로 알려져 있다. 이에 저자들은 항산화제로 알려진 알파리포산이 혈관 평활근세포의 증식속도와 이주에 미치는 영향과 PAI1발현에 미치는 영향을 조사하였고 이와 동시에 백서의 혈관 손상 모델을 이용하여 알파리포산이 신생내막 증식을 억제시킬 수 있는지를 알아보았다. 방법:In vitro 실험은 백서의 대동맥 평활근세포를 고농도의 포도당(22mM)과 100nM의 안지오텐신Ⅱ로 4시간 배양하였다. 알파리포산을 처리 후, PAI1 mRNA의 발현을 보기 위해 노던 블롯을 시행하였고 평활근세포의 유주능과 증식속도에 미치는 영향을 보기 위해 성장속도의 유주능 분석을 시행하였다. 또한 평활근세포의 유주능에 NFμB 경로가 미치는 영향을 보기 위해 겔 지연 분석과 NFμB 보고 유전자 분석을 시행하였다. In vivo 실험으로 백서의 혈관손상 모델을 이용하여 알파리포산 처리 후 혈관의 신생내막 두께를 비교하였다. 결과:In vitro 실험에서 고농도 포도당과 안지오텐신Ⅱ에 의해 유도된 PAI1 mRNA 발현증가는 알파리포산 처리에 의하여 용량에 비례하여 억제되었다(p<0.05). 알파리포산 처리로 혈관 평활근세포의 유주능은 유의하게 억제되었으나(p<0.01),증식속도는 유의하게 억제되지 않았다. 또한 알파리포산 처리로 NFμB 발현도 유의하게 억제되었다(p<0.01). In vivo 실험에서 알파리포산을 주입한 군에서 혈관손상에 의한 신생내막의 증식이 유의하게 억제되는 것을 보여주었다(p<0.01). 결론:알파리포산은 백서의 대동맥 평활근 세포의 증식속도는 억제하지 못했으나 유주능은 유의하게 억제하였으며, 혈관 손상 모델에서 신생 내막 증식도 유의하게 억제함을 확인할 수 있었다. 이러한 작용은 알파리포산에 의한 NFμB 경로의 억제와 연관이 있는 것으로 사료된다. Background : Exposure to large amounts of glucose causes a characteristic dysfunction and morphologic changes of the endothelium by an increased production of reactive oxygen species(ROS) in diabetes. The plasminogen activator inhibitor-1(PAI-1), which modulates fibrinolysis and cell migration may influence proteolysis and neointimal formation in vascular smooth muscle cells(VSMC). Antioxidants have been proposed to inhibit multiple proatherogenic events. This study investigated the effect of α-lipoic acid on PAI-1 expression and VSMC proliferation and migration both in vivo and in vitro. Methods : In the in vitro study, cultured rat aortic smooth muscle cells(RASMC) were incubated in a medium containing high glucose (22 mM) and 100 nM angiotensin Ⅱ for 4 hour. After α-lipoic acidtreatment, a -migration and growth assay of the RASMC, and a gelmobility shift assay and reportergene analysis for nuclear factor- иB(NF- иB) and northern blot analysis for PAI-1 were performed. In the in vivo study, the effect of α-lipoic acid on neointimal hyperplasia in a rat carotid balloon injury model was evaluated. Results : RASMC migration was inhibited significantly by α-lipoic acid (p<0.01), but their prolife ration was not inhibited. The NF-иB DNA binding activity and NF-иB promoter activity was inhibited by α-lipoic acid significantly (p<0.01). α-lipoic acid inhibited PAI-1 mRNA expression by high glucose and angiotensin Ⅱ in dose dependent manner (p<0.05). In the rat carotid artery balloon injury model, neointimal formation was reduced by α-lipoic acid treatment in a dose dependent manner significantly (p<0.01). Conclusion : α-lipoic acid suppresses migration, but not prolife ration in RASMC. α-lipoic acid also reduce neointima formation in a rat carotid balloon injured model. This effect might be related to the blocking of NF-иB which increase the expression of the genes associated with atherosclerosis including TNF-α, IL-1, IL-6, endothelin-1, MCP-1, VCAM-1, ICAM-1, E-selectin, tissue factor(J Kor Diabetes Asso 25:446~459, 2001).
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Fabrication and mechanical properties of silicon nitride laminate composites
신동우,Hai Guo 한양대학교 세라믹연구소 2005 Journal of Ceramic Processing Research Vol.6 No.3
A compact material with a laminated structure similar to the structure of nacre has been successfully made using a procedure described in this paper. This laminated structural design provides high-fracture toughness to Si3N4/BN-Al2O3 ceramic composites. The dense Si3N4 laminate (99.6% of theoretical density) had a flexural strength of 500 Mpa and a fracture toughness of 15 MPa·m1/2. The introduction of reinforcement (15 wt% SiC whiskers) within the Si3N4/BN-Al2O3 laminated ceramic composites further increases the strength and fracture toughness values to 650 MPa and 20 MPa·m1/2, respectively.
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