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      • Clinical and histopathological study of Charcot-Marie-Tooth neuropathy with a novel S90W mutation in BSCL2.

        Choi, B-O,Park, M-H,Chung, K W,Woo, H-M,Koo, H,Chung, H-K,Choi, K-G,Park, K D,Lee, H J,Hyun, Y S,Koo, S K Oxford University Press 2013 Neurogenetics Vol.14 No.1

        <P>The objective of the study was to investigate the disease-causing mutation in an autosomal dominant Charcot-Marie-Tooth disease type 2 family and examine the clinical and histopathological evaluation. We enrolled a family of Korean origin with axonal Charcot-Marie-Tooth disease neuropathy (FC305; 13 males, six females) and applied genome-wide linkage analysis. Whole exome sequencing was performed for two patients. In addition, sural nerve biopsies were obtained from two patients. Through whole exome sequencing, we identified an average of 20,336 coding variants from two patients. We also found evidence of linkage mapped to chromosome 11p11-11q13.3 (LOD score of 3.6). Among these variants in the linkage region, we detected a novel p.S90W mutation in the Berardinelli-Seip congenital lipodystrophy 2 (BSCL2) gene, after filtering 31 Korean control exomes. Our p.S90W patients had frequent sensory disturbances, pyramidal tract signs, and predominant right thenar muscle atrophy in comparison with reported p.S90L patients. The phenotypic spectra were wide and demonstrated intrafamilial variability. Two patients with different clinical features underwent sural nerve biopsies; the myelinated fiber densities were increased slightly in both patients, which differed from two previous case reports of BSCL2 mutations (p.S90L and p.N88S). This report expands the variability of the clinical spectrum associated with the BSCL2 gene and describes the first family with the p.S90W mutation.</P>

      • KCI등재
      • 제주재래흑돼지 자돈의 수유행동에 관한 연구

        강민수,고봉석,조성환,현종훈,김현호,현승구,고기정,박미혜 濟州大學校 農科大學 動物科學硏究所 1999 動物科學論叢 Vol.14 No.1

        This study was conducted to the suckling behavior on the growth of Cheju native black piglet during the suckling period. The data for this study were obtained from 24 heads of Cheju native black piglet(female 15 and male 9). The traits studied were composition of milk and suckling behaviour of piglets. The results obtained in this study are summaried as follows; 1. The suckling duration of piglets were 126.4 min. after 1 wk, 93.2 min. after 3 wk, 72.4 min. after 6 wk, respectively. There were significant difference between weeks(p<0.05). The suckling interval of piglets were 32.7 min. after 1 wk, 44.6 min. after 3 wk, 47.3 min. after 6 wk, respectively. There were significant difference between weeks(p<O.O5). The suckling frequency of piglets were 19.6 times after 1 wk, 14.5 times after 3 wk, 13.9 times after 6 wk, respectively. There were significant difference between weeks(p<O.O5). 2. The resting duration of piglets were 963 min. after 1 wk, 1023 min. after 3 wk, 966 min. after 6 wk, respectively. There were not significant difference between weeks(p<0.05). The playing duration of piglets were wk, 191 min. after 3 wk, 215 min. after 6 wk, respectively. There were not were 20 min. after 3 wk, 48 min. after 5wk, 62 min. after 6 wk, respectively There were significant difference between weeks(p<0.05). 3. The urination frequent). of piglets were 13.9 times after 1 ivk, 8.0 times after 3 wk, 1.7 times after 6 wk, respectively. There were significant dfference between weeks(p(O.01). The defecation frequency of piglets were 0.9 times after 1 wk, 1.8 times after 3 wk, 3.4 times after 6 wk, respectively. There tvere significant dfference between weeks(p<0.0l). The drinking water frequency of piglets were 3.3 times after 1wk, 48 times after 3 wk, 10.7 times after 6 wk. respectively. There were significant dfference between weeks(p<0.0l). 4. Protein content of native sows milk was considerablely higher than that of the introduced sows milk but the fat and sugar content in the milk were lower than those of introduced sows milk. The results of suckling behaviour and litter performance study of Cheju native piglets may contribute to the effective breeding and feedmg management of Cheju native black swine production.

      • 제주재래흑돼지 자돈의 성장에 관한 연구

        강민수,고봉석,조성환,현종훈,김현호,현승구,고기정,박미혜 濟州大學校 農科大學 動物科學硏究所 1999 動物科學論叢 Vol.14 No.1

        This study was conducted to the suckling behavior on the growth of Cheju native black piglet during the suckling period. The data for this study were obtained from 24 heads of Cheju native black piglet(fema1e 15 and male 9). The traits studied were composition of milk, body weight and comformation of piglets. The results obtained in this study are summaried as follows; 1. The composition of clostrum were 80.11 % water, 19.89 % solids, 7.46 fat, 8.95 % protein, 3.29 % lactose, 0.29% minerals, pH value was 6.42. The composition of normal milk were 83.81 % water, 16.19 solids, 5.79 % fat, 5.34 % protein, 4.28 % lactose, 0.77 % minerals, pH value was 5.49. 2. The body weight in female and male piglets, respectively was 1.01 and 1.17 kg at birth, 4.64 and 5.36 kg at 3 wk, 9.49 and 11.04 kg at 6 wk of age. There difference between male and female was significant(p<O.&). 3. The withers height in female and male was 15.47 and 16.43 cm at birth, 23.91 and 24.86cm for 3 wk, 30.47 and 32.14 cm for 6 wk old piglets, respectively. There difference between male and female was significant(p<0.05). The back height in female and male was 16.19 and 16.93 cm at birth, 25.34 and 26.36 an 16.50 after 3 wk, 32.06 and 33.71 cm after 6 wk, respectively. There dfference between male and female was significant(p<O.B). The croup height of female and male was 15.78 and 16.50cm at birth, 32.16 and 3 . 1 4 cm after 6 wk, respectively. There dfference between male and female ivas significant(p<O.05). 4. The body length in female and male piglets was 18.69 and 19.79 cm at birth, 32.22 and 33.86 cm after 3 wk, 42.59 and 43.07 cm after 6 wk, respectively. There dfference betiveen male and female ignificant(p<0.05). The chest girth in female and male piglets was 22.28 and 23.43 cm at birth, 38.31 and 39.6.1 cm after 3 wk. 37.81 and 50.00 cm after 6 wk, respectively. There dfference between male and female was significant(p<0.05). 5. The chest width in female and male was 513 and 5.36 cm at birth, 8.93 and 10.07 cm after 3 wk, 12.09 and 13.00 cm after 6 wk, respectively. There dfference between male and female was not significant(p<0.05). The pine width in female and male was 2.03 and 2.71 cm at birth, 3.97 and 4.93 cm after 3 wk, 5.88 and 6.57 cm after 6 wk, respectively. There dfference between male and female was significant(p<0.05). 6. The shin circumference in female and male was 5.03 and 5.64 cm at birth, 7.34 and 7.79 cm after 3 wk, 8.86 and 9.36 cm after 6 wk, respectively. There difference between male and female was significant(p<0.05). The head length in female and male was 6.84 and 7.43 cm at birth, 9.87 and 10.21 cm after 3 wk, 12.41 and 13.00 cm after 6 wk, respectively. There dfference between male and female was not significant(p<0.05). The head width in female and male was 4.16 and 4.57 cm at birth, 5.97 and 6.14 cm after 3 wk, 7.09 and 7.71 cm after 6 wk, respectively. There dfference between male and female was not significant(p<0.05). 7. The ear length in female and male was 5.56 and 5.86 cm at birth, 8.47 and 9.14 after 3 wk, 10.78 and 11.50 cm after 6 wk, respectively. There dfference between male and female was significant(p<0.05). The ear width in female and male was 4.19 and 4.64 cm at birth, 6.03 and 6.43 cm after 3 wk, 8.09 and 8.64 cm after 6 wk, respectively. There dfference between male and female was significant(p<0.05). The tail length in female and male was 7.25 and 7.29cm at birth, 10.38 and 10.50 cm after 3 wk, 13.88 and 14.43 cm after 6 wk, respectively. There dfference between male and female was not significant(p<0.05).

      • KCI등재SCIESCOPUS

        Enhanced efficiency and stability of polymer solar cells using solution-processed nickel oxide as hole transport material

        Parthiban, S.,Kim, S.,Tamilavan, V.,Lee, J.,Shin, I.,Yuvaraj, D.,Jung, Y.K.,Hyun, M.H.,Jeong, J.H.,Park, S.H. ELSEVIER 2017 Current Applied Physics Vol.17 No.10

        <P>Solution-processed nickel oxide (s-NiOx) was synthesized for use as hole-transport layers (HTLs) in the fabrication of polymer solar cell (PSC) devices. The s-NiOx thin-films were deposited using spin-coating and post-annealed at 300 degrees C, 400 degrees C, or 500 degrees C. With increased annealing temperature, the nickel acetate precursor decomposes more fully and forms s-NiOx films that show larger crystalline grain sizes with lower root mean square surface roughness. Bulk heterojunction solar cells fabricated with the new random polymer RP(BDT-PDBT) and [6,6]-phenyl-C-70-butyric acid methyl ester (PC70BM) using s-NiOx as HTLs exhibit a 4.46% enhancement in power conversion efficiency and better stability compared to conventional PSCs using poly (3,4-ethylenedioxythiophene):poly(styrene sulfonate) as HTLs. We believe that the solution-processable and highly stable s-NiOx could be a potential alternative for functional interface materials in optoelectronic devices. (C) 2017 Elsevier B.V. All rights reserved.</P>

      • KCI등재
      • SCISCIESCOPUS

        Evaluation of the Pentacam Ray Tracing Method for the Measurement of Central Corneal Power After Myopic Photorefractive Keratectomy

        Oh, Jong-Hyun,Kim, Sung Hyun,Chuck, Roy S.,Park, Choul Yong Masson Pub. USA 2014 Cornea Vol.33 No.3

        PURPOSE:: The study evaluated the ray tracing method [total corneal refractive power (TCRP)] in a Pentacam apparatus (Oculus, Wetzlar, Germany) for postoperative keratometry measurement after myopic photorefractive keratectomy (PRK). METHODS:: Manifest refraction (MR) and Pentacam analyses were performed preoperatively and at 6 months postoperatively after the PRK (STAR S4 IR CustomVue; Abbott Medical Optics/Visx) in 49 right eyes from 49 patients (age, 25.42 ± 3.51 years). Postoperative corneal power was calculated using the clinical history method (CHM) and compared with postoperatively measured simulated keratometry (simK), true net power (TNP) at 3 mm, and pupil-centered TCRP at the center, 1, 3, and 4 mm (TCRP0, TCRP1, TCRP3, and TCRP4). Vertex-distance-adjusted refractive change (delta-MR) at the corneal plane was also compared with various keratometric changes (delta-K). RESULTS:: Postoperative TCRP0, TCRP1, TCRP3, and TCRP4 showed no significant difference compared with that of the CHM. Postoperative simK was significantly higher than that of the CHM, whereas the TNP was significantly lower compared with that of the CHM. The delta-Ks measured by simK, TNP, and TCRPs were significantly smaller than delta-MR, and delta-TCRP4 showed the least difference [mean ± SD, 0.28 ± 0.55 diopters (D)] with delta-MR. The 95% limit of agreement between delta-MR and delta-TCRP4 was −0.85 to 1.31 D. The difference between delta-TCRP4 and delta-MR was <0.5 D in 55.1% and <1.0 D in 87.8% of the eyes studied. CONCLUSIONS:: Although postoperative TCRPs showed no significant difference with CHM, delta-MR was still underestimated after myopic PRK.

      • KCI등재

        Enzymatic Saccharification of Salix viminalis cv. Q683 Biomass for Bioethanol Production

        Hak-Gon Kim,Hyun-Jin Song,Mi-Jin Jeong,Seon-Jeong Sim,Dong-Jin Park,Jae-Kyung Yang,Seok-Bong Yoo,Jin-Ki Yeo,Chandrakant S. Karigar,Myung-Suk Choi 강원대학교 산림과학연구소 2011 Journal of Forest Science Vol.27 No.3

        The possibility of employing biomass of Salix viminalis cv. Q683 as a resource of bio-energy was evaluated. The chemical analysis of S. viminalis cv. Q683 leaf biomass showed components such as, extractives (2.57%), lignin (39.06%), hemicellulose (21.61%), and cellulose (37.83%), whereas, its stem was composed of extractives (1.67%), lignin (23.54%), hemicellulose (33.64%), and cellulose (42.03%). The biomass of S. viminalis cv. Q683 was saccharified using two enzymes celluclast and viscozyme. The saccharification of S. viminalis cv. Q683 biomass was influenced by enzymes and their strengths. The optimal enzyme combination was found to be celluclast (59 FPU/g substrate) and viscozyme (24 FBG/g substrate). On saccharification the glucose from leaf and stem biomass was 7.5g/L and 11.7g/L, respectively after 72 hr of enzyme treatment. The biomass and enzyme-treated biomass served as the feedstock for ethanol production by fermentation. The ethanol production from stem and leaf biomass was 5.8 g/L and 2.2 g/L respectively, while the fermentation of the enzymatic hydrolysates yielded 5 g/L to 8 g/L bioethanol in 72 hours.

      • KCI등재

        Enzymatic Saccharification of Salix viminalis cv. Q683 Biomass for Bioethanol Production

        Kim, Hak-Gon,Song, Hyun-Jin,Jeong, Mi-Jin,Sim, Seon-Jeong,Park, Dong-Jin,Yang, Jae-Kyung,Yoo, Seok-Bong,Yeo, Jin-Ki,Karigar, Chandrakant S.,Choi, Myung-Suk Institute of Forest Science 2011 Journal of Forest Science Vol.27 No.3

        The possibility of employing biomass of Salix viminalis cv. Q683 as a resource of bio-energy was evaluated. The chemical analysis of S. viminalis cv. Q683 leaf biomass showed components such as, extractives (2.57%), lignin (39.06%), hemicellulose (21.61%), and cellulose (37.83%), whereas, its stem was composed of extractives (1.67%), lignin (23.54%), hemicellulose (33.64%), and cellulose (42.03%). The biomass of S. viminalis cv. Q683 was saccharified using two enzymes celluclast and viscozyme. The saccharification of S. viminalis cv. Q683 biomass was influenced by enzymes and their strengths. The optimal enzyme combination was found to be celluclast (59 FPU/g substrate) and viscozyme (24 FBG/g substrate). On saccharification the glucose from leaf and stem biomass was 7.5g/L and 11.7g/L, respectively after 72 hr of enzyme treatment. The biomass and enzyme-treated biomass served as the feedstock for ethanol production by fermentation. The ethanol production from stem and leaf biomass was 5.8 g/L and 2.2 g/L respectively, while the fermentation of the enzymatic hydrolysates yielded 5 g/L to 8 g/L bioethanol in 72 hours.

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