• Title/Summary/Keyword: SMN

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Extradiol Cleavage of Two-ring Structures of Biphenyl and Indole Oxidation by Biphenyl Dioxygenase in Commamonas Acidovorans

  • On, Hwa-Young;Lee, Na-Ri;Kim, Young-Chang;Kim, Chi-Kyung;Kim, Young-Soo;Park, Yong-Keun;Ka, Jong-Ok;Lee, Ki-Sung;Min, Kyung-Hee
    • Journal of Microbiology and Biotechnology
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    • v.8 no.3
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    • pp.264-269
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    • 1998
  • Commamonas acidovorans SMN4 showed wide growth substrate spectra for various aromatic hydrocarbons. Strain SMN4 was able to grow on biphenyl producing a meta-cleavage compound, yellow 2-hydroxy-6-oxophenylhexa-2,4-dienoic acid with a spray of 2,3-dihydroxybiphenyl, while it also grew on catechol, developing yellow 2- hydroxymucoic semialdehyde with a spray of 100 mM catechol. Thus these results indicate that two-ring structures of biphenyl were cleaved by meta-mode in upper and lower pathways. Strain SMN4 metabolized various substituted biphenyl compounds and xylene to the corresponding benzoate derivatives through oxidation of the ring structures. It was clearly shown that biphenyl can be a common inducer in the oxidation of biphenyl and 2,3-dihydroxybiphenyl. Various compounds were examined for their suitability to serve as substrates for indole oxidation, indicating that biphenyl, benzoate, and succinate are quite good inducers of indigo production due to the activity of biphenyl dioxygenase. This results suggest that indigo formation is by means of the combined activities of biphenyl dioxygenase and tryptophanase.

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Prenatal diagnosis of the spinal muscular atrophy type I using genetic information from archival slides and paraffin-embedded tissues

  • Choi, Soo-Kyung;Cho, Eun-Hee;Kim, Jin-Woo;Park, So-Yeon;Kim, Young-Mi;Ryu, Hyun-Mee;Kang, Inn-Soo;Jun, Jung-Young;Chi, Je-G.
    • Journal of Genetic Medicine
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    • v.2 no.2
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    • pp.53-57
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    • 1998
  • Spinal muscular atrophy (SMA) type I is a common severe autosomal recessive inherited neuromuscular disorder that has been mapped to chromosome 5q11.2-13.3. The survival motor neuron (SMN) gene, a candidate gene, is known to be deleted in 96% of patients with SMA type I. Presently, PCR and single strand conformation polymorphism (PCR-SSCP) analyses have been made possible for application to both archival slides and paraffin-embedded tissues. Archival materials represent valuable DNA resources for genetic diagnosis. We applied these methods for the identification of SMN gene of SMA type I in archival specimens for the prenatal diagnosis. In this study, we performed the prenatal diagnosis with chorionic villus sampling (CVS) cells on two women who had experienced neonatal death of SMA type I. DNA extraction was done from archival slide and tissue materials and PEP-PCR was performed using CVS cells. In order to identify common deletion region of SMN and neuronal apoptosis-inhibitory protein (NAIP) genes, cold PCR-SSCP and PCR-restriction site assay were carried out. Case 1 had deletions of the exons 7 and 8, and case 2 had exon 7 only on the telomeric SMN gene. Both cases were found to be normal on NAIP gene. These results were the same for both CVS and archival biopsied specimens. In both cases, the fetuses were, therefore, predicted to be at very high risk of being affected and the pregnancy were terminated. These data clearly demonstrate that archival slide and paraffin-embedded tissues can be a valuable source of DNA when the prenatal genetic diagnosis is needed in case any source for genetic analysis is not readily available due to previous death of the fetus or neonate.

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Dielectric Properties of Sr(Mg1/3Nb1/3)O3-Ba(Mg1/3Nb2/3)O3-Ba(Mg1/3Ta2/3)O3 Solid Solution (Sr(Mg1/3Nb1/3)O3-Ba(Mg1/3Nb2/3)O3-Ba(Mg1/3Ta2/3)O3고용체의 유전성 (복합 Perovskite구조를 갖는 세라믹스의 유전성))

  • 윤기현;정범준;김응수;강동헌
    • Journal of the Korean Ceramic Society
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    • v.25 no.6
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    • pp.639-644
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    • 1988
  • The physical and dielectric properties of complex perovskite compound Sr(Mg1/3Nb1/3)O3-Ba(Mg1/3Nb2/3)O3-Ba(Mg1/3Ta2/3)O3(BMT) system were investigated as a function of composition. As the mole ratio of BMN was increased, lattice parameter ratio c/a was slightly increased, and density was increased in SMN-BMN system. However, in BMN-BMT system, lattice parameter ratio c/a and density were decreased with increasing the mole ratio of BMN. Dielectric constant, dielectric loss at $25^{\circ}C$ and 100kHz, and temperature coefficient of resonant frequency, the dependence of temperature in capacitance were increased with increasing the mole ratio of BMN in SMN-BMN-BMT system. These result can be explained according to the degree of order=disorder and dielectric constant.

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Physical Properties of $Ba_{1-x}Sr_x(Mg{1/3}Nb_{2/3})O_3$Ceramics ($Ba_{1-x}Sr_x(Mg{1/3}Nb_{2/3})O_3$ 세라믹스의 물리적 특성)

  • 김부근;김재윤;김강언;정수태;조상희
    • Proceedings of the Korean Institute of Electrical and Electronic Material Engineers Conference
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    • 1999.05a
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    • pp.325-328
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    • 1999
  • The physical and electrical properties of $\textrm{Ba}_1$ $_{x}\textrm{Sr}_{x}$($\textrm{Mg_{1/3}Nb_{2/3}}$)$\textrm{O}_3$ (x =0, 0.2, 0.4, 0.6, 0.8, 1.0) ceramics were investigated. The Bal $_{x}\textrm{Sr}_{x}$($\textrm{Mg_{1/3}Nb_{2/3}}$)$\textrm{O}_3$ systems were shown that the hexagonally ordered superlattices were increased with increasing x values. The relative densities of all samples were over 97% theoretical densities. The dc resistivities of samples were $10^{13}$ - $10^{14}$$\Omega\textrm{cm}$at room temperature, these values were nearly constant at 130(x=0)-$230^{\circ}C$ (x=l). However, the resistivities of samples decreased rapidly above those temperature and their activation energies were from 1.0 to 1.52 eV. The relative dielectric constant was 33(BMN) and 30.6(SMN) respectively. And the highest value was shown at x=0.4 and the value was 34.3. The temperature coefficient of dielectric constant was -61 ppm/$^{\circ}C$(BMN) and 79 ppm/$^{\circ}C$ (SMN) respectively.

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Purification and Characterization of 2,3-Dihydroxybiphenyl 1,2- Dioxygenase from Comamonas sp.

  • Lee Na Ri;Kwon Dae Young;Min Kyung Hee
    • Proceedings of the Microbiological Society of Korea Conference
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    • 2001.11a
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    • pp.16-25
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    • 2001
  • A genomic library of biphenyl-degrading strain Comamonas sp. SMN4 was constructed by using the cosmid vector pWE15 and introduced into Escherichia coli. Of 1,000 recombinant clones tested, two clones that expressed 2,3-dihydroxybiphenyl 1,2-dioxygenase activity were found (named pNB 1 and pNB2). From pNB1 clone, subclone pNA210, demonstrated 2,3-dihydroxybiphenyl 1,2-dioxygenase activity, is isolated. 2,3-Dihydroxybiphenyl 1,2-dioxygenase (23DBDO, BphC) is an extradiol-type dioxygenase that involved in third step of biphenyl degradation pathway. The nucleotide sequence of the Comamonas sp. SMN4 gene bphC, which encodes 23DBDO, was cloned into a plasmid pQE30. The His-tagged 23DBDO produced by a recombinant Escherichia coli, SG 13009 (pREP4)(pNPC), and purified with a Ni-nitrilotriacetic acid resin affinity column using the His-bind Qiagen system. The His-tagged 23DBDO construction was active. SDS-PAGE analysis of the purified active 23DBDO gave a single band of 32 kDa; this is in agreement with the size of the bphC coding region. The 23DBDO exhibited maximum activity at pH 9.0. The CD data for the pHs, showed that this enzyme had a typical a-helical folding structures at neutral pHs ranged from pH 4.5 to pH 9.0. This structure maintained up to pH 10.5. However, this high stable folding strucure was converted to unfolded structure in acidic region (pH 2.5) or in high pH (pH 12.0). The result of CD spectra observed with pH effects on 23DBDO activity, suggested that charge transition by pH change have affected change of conformational structure for 23DBDO catalytic reaction. The $K_m$ for 2,3-dihydroxybiphenyl, 3-metylcatechol, 4-methylcatechol and catechol was 11.7 $\mu$M, 24 $\mu$M, 50 mM and 625 $\mu$M.

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Surgical Results of Selective Median Neurotomy for Wrist and Finger Spasticity

  • Kwak, Kyung-Woo;Kim, Min-Su;Chang, Chul-Hoon;Kim, Sang-Woo;Kim, Seong-Ho
    • Journal of Korean Neurosurgical Society
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    • v.50 no.2
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    • pp.95-98
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    • 2011
  • Objective : This study aimed to evaluate the surgical outcomes of selective median neurotomy (SMN) for spastic wrist and fingers. Methods : We studied 22 patients with wrist and finger spasticity refractory to optimal oral medication and physical therapy. The authors evaluated spasticity of the wrist and finger muscles by comparing preoperative states with postoperative states using the modified Ashworth scale (MAS). We checked patients for changes in pain according to the visual analog scale (VAS) and degree of satisfaction based on the VAS. Results : The preoperative mean MAS score was $3.27{\pm}0.46$ ($mean{\pm}SD$), and mean MAS scores at 3, 6, and 12 months after surgery were $1.82{\pm}0.5$, $1.73{\pm}0.7$, and $1.77{\pm}0.81$ ($mean{\pm}SD$), respectively. On the last follow-up visit, the mean MAS score measured $1.64{\pm}0.9$ ($mean{\pm}SD$). Wrist and finger spasticity was significantly decreased at 3, 6, and 12 months after the operation (p<0.01). The preoperative mean pain VAS score was $5.85{\pm}1.07$ ($mean{\pm}SD$), and the mean pain VAS score on the last follow-up visit after surgery was $2.28{\pm}1.8$ ($mean{\pm}SD$). Compared with the preoperative mean pain VAS score, postoperative mean pain VAS score was decreased significantly (p<0.01). On the basis of a VAS ranging from 0 to 100, the mean degree of patient satisfaction was $64.09{\pm}15.93$ ($mean{\pm}SD$, range 30-90). Conclusion : The authors propose SMN as a possible effective procedure in achieving useful, long-lasting tone and in gaining voluntary movements in spastic wrists and fingers with low morbidity rates.

Differences in Large-scale and Sliding-window-based Functional Networks of Reappraisal and Suppression

  • Jun, Suhnyoung;Lee, Seung-Koo;Han, Sanghoon
    • Science of Emotion and Sensibility
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    • v.21 no.3
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    • pp.83-102
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    • 2018
  • The process model of emotion regulation suggests that cognitive reappraisal and expressive suppression engage at different time points in the regulation process. Although multiple brain regions and networks have been identified for each strategy, no articles have explored changes in network characteristics or network connectivity over time. The present study examined (a) the whole-brain network and six other resting-state networks, (b) their modularity and global efficiency, which is an index of the efficiency of information exchange across the network, (c) the degree and betweenness centrality for 160 brain regions to identify the hub nodes with the most control over the entire network, and (d) the intra-network and inter-network functional connectivity (FC). Such investigations were performed using a traditional large-scale FC analysis and a relatively recent sliding window correlation analysis. The results showed that the right inferior orbitofrontal cortex was the hub region of the whole-brain network for both strategies. The present findings of temporally altering functional activity of the networks revealed that the default mode network (DMN) activated at the early stage of reappraisal, followed by the task-positive networks (cingulo-opercular network and fronto-parietal network), emotion-processing networks (the cerebellar network and DMN), and sensorimotor network (SMN) that activated at the early stage of suppression, followed by the greater recruitment of task-positive networks and their functional connection with the emotional response-related networks (SMN and occipital network). This is the first study that provides neuroimaging evidence supporting the process model of emotion regulation by revealing the temporally varying network efficiency and intra- and inter-network functional connections of reappraisal and suppression.

Two cases of spinal muscular atrophy type 1 with extensive involvement of sensory nerves (광범위한 감각신경 침범을 동반한 척수성 근위축증 2예)

  • Lee, Ran;Chung, Sochung;Koh, Sung-Eun;Lee, In Kyu;Lee, Jongmin
    • Clinical and Experimental Pediatrics
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    • v.51 no.12
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    • pp.1350-1354
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    • 2008
  • Spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by diffuse proximal and distal weakness due to deletion of the survival motor neuron (SMN) gene localized on chromosome 5 (5q11.2-13.3). SMA has been considered as a pure lower motor neuron disorder, and a definitive diagnosis can be established by molecular genetic testing. Here, we describe two patients with severe hypotonia and frequent aspirations at early infancy. Nerve conduction studies showed more extensive sensory involvement in these patients diagnosed to have SMA by genetic study than in classical cases of SMA. To the best of our knowledge, this is the first report of SMA Type 1 with sensory nerve involvement in Korea.

Dissimilar Friction Welding for Pinion Gear and Its Real Time AE Evaluation (자동차용 피니온 기어재의 이종재 마찰용접과 실시간 AE평가)

  • Oh, Sae-Kyoo;Son, Deog-Soo;Yang, Hyung-Tae
    • Journal of Ocean Engineering and Technology
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    • v.13 no.4 s.35
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    • pp.105-111
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    • 1999
  • This study aimed not only to develop the optimization of dissimilar friction welding of pinion gear of a motor vehicle steels of SCM415 and SMn443, but also to develop the application technique of the acoustic emission to accomplish an in-process real-time quality(such as strength and toughness) evaluation during friction welding of the pinion gear by the AE technique.

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