• Title/Summary/Keyword: $Q_C^{-1}$

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Improved Acroparesthesia During Enzyme Replacement Therapy in a Patient Lately Diagnosed with Fabry Disease (진단이 지연된 Fabry 병 환자에서 효소대체요법을 통한 사지 말단 동통의 호전을 보인 1례)

  • Yang, Aram;Kim, Jinsup;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.17 no.3
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    • pp.92-95
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    • 2017
  • Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

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Effects of Experimental Warming on Growth of Quercus variabilis Seedlings (실외 실험적 온난화 처리가 굴참나무 묘목의 생장에 미치는 영향)

  • Lee, Sun Jeoung;Han, Saerom;Yoon, Tae Kyung;Chung, Haegeun;Noh, Nam Jin;Jo, Wooyong;Park, Chan-Woo;Ko, Suin;Han, Seung Hyun;Son, Yowhan
    • Journal of Korean Society of Forest Science
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    • v.101 no.4
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    • pp.722-728
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    • 2012
  • Climate change affects all biological processes in terrestrial ecosystems including photosynthesis, plant growth and productivity. This study was conducted to investigate the effects of experimental warming on the growth of Quercus variabilis seedlings. One-year-old Q. variabilis seedlings were planted in control and warmed plots in April 2010. The air temperature of warmed plots was increased by $3^{\circ}C$ compared to control plots using the infrared lamp from November 2010. Shoot height and root collar diameter were measured in March 2011 and June 2012, respectively, and aboveground and belowground biomass were also measured in March 2011 and 2012, respectively. Shoot height and root collar diameter were significantly higher in warmed plots than in control plots, except for root collar diameter in March 2011. Increment (mm) of shoot height and root collar diameter were also higher in warmed plots ($529{\pm}30$, $5.6{\pm}0.5$) than in control plots ($464{\pm}28$, $4.5{\pm}0.4$). However, there were no significant differences between warmed and control plots except for root collar diameter. Increment (g/year) of total, aboveground and belowground biomass were higher in warmed plots ($36.88{\pm}6.52$, $11.91{\pm}3.44$, $24.97{\pm}3.73$) than in control plots ($30.59{\pm}5.51$, $8.73{\pm}1.66$, $21.86{\pm}3.88$), however, the differences were not statistically significant. Higher seedling growth and biomass of warmed plots might be related to the enhanced net photosynthetic rates in spring and the extended growing season.

Expression Profiling of MLO Family Genes under Podosphaera xanthii Infection and Exogenous Application of Phytohormones in Cucumis melo L. (멜론 흰가루병균 및 식물 호르몬 처리하에서 MLO 유전자군의 발현검정)

  • Howlader, Jewel;Kim, Hoy-Taek;Park, Jong-In;Ahmed, Nasar Uddin;Robin, Arif Hasan Khan;Jung, Hee-Jeong;Nou, III-Sup
    • Journal of Life Science
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    • v.26 no.4
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    • pp.419-430
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    • 2016
  • Powdery mildew disease caused by Podosphaera xanthii is a major concern for Cucumis melo production worldwide. Knowledge on genetic behavior of the related genes and their modulating phytohormones often offer the most efficient approach to develop resistance against different diseases. Mildew Resistance Locus O (MLO) genes encode proteins with seven transmembrane domains that have significant function in plant resistance to powdery mildew fungus. We collected 14 MLO genes from ‘Melonomics’ database. Multiple sequence analysis of MLO proteins revealed the existence of both evolutionary conserved cysteine and proline residues. Moreover, natural genetic variation in conserved amino acids and their replacement by other amino acids are also observed. Real-time quantitative PCR expression analysis was conducted for the leaf samples of P. xanthii infected and phyto-hormones (methyl jasmonate and salicylic acid) treated plants in melon ‘SCNU1154’ line. Upon P. xanthii infection using 7 different races, the melon line showed variable disease reactions with respect to spread of infection symptoms and disease severity. Three out of 14 CmMLO genes were up-regulated and 7 were down-regulated in leaf samples in response to all races. The up- or down-regulation of the other 4 CmMLO genes was race-specific. The expression of 14 CmMLO genes under methyl jasmonate and salicylic acid application was also variable. Eleven CmMLO genes were up-regulated under salicylic acid treatment, and 7 were up-regulated under methyl jasmonate treatments in C. melo L. Taken together, these stress-responsive CmMLO genes might be useful resources for the development of powdery mildew disease resistant C. melo L.

A Development and Application of the Landscape Evaluation Model Based on the Biotope Classification (비오톱 유형분류를 기반으로 한 경관평가 모형개발 및 적용)

  • Park, Cheon-Jin;Ra, Jung-Hwa;Cho, Hyun-Ju;Kim, Jin-Hyo;Kwon, Oh-Sung
    • Journal of the Korean Institute of Landscape Architecture
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    • v.40 no.4
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    • pp.114-126
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    • 2012
  • The purpose of this study is to find ways of the view evaluation of biotope classification before development by selecting an area, which is as large as about $10.0km^2$ around Non Gong Up, Auk Po Myun, Dalsung Gun, Daugu where the large project has been planned, as a subject of this study. The results of this study are as follows. Because of the classification of biotope, there are 23 kinds of types that are subdivided into 140 types. Three surveys for selecting the assessment indicators were performed. The first survey analyzed the importance of 22 selected assessment indicators based on the evaluation of an existing literature review and on the spot research. The second survey performed factor analysis and reclassified the value indicators. The third survey computed additive values of the selected assessment indicators. It used a method of standardizing the average importance of indicators by making their sum equal by 10. Theses additive values were then multiplied by each grade of indicators in order to make a final evaluation. The number of assessment indicators finally selected through the survey of asking specialist is vitality elements, visual obstructs elements etc 19. According to the result of evaluation of 1st, 1 grade spaces which especially valuable is analyzed that 7 spaces, 2 grade spaces for 4, 3 grade spaces for 5, 4 grade space for 2, 5 grade space for 5. Because of the evaluation of 2st, 1 grade spaces which especially valuable(1a, 1b) is analyzed that 15 spaces, 2 grade spaces which valuable is analyzed that 28 space. As the evaluation of site suitability model of this study couldn't have high applicability to other similar area because of having only one site as a subject, it is needed to do synthesize and standardization of various examples to have higher objectivity later.

Effects of Physical Parameters on Water Quality in Agricultural Reservoirs (농업용 저수지의 물리적 인자가 수질에 미치는 영향)

  • Jeon, Ji-Hong;Ham, Jong-Hwa;Kim, Ho-Il;Hwang, Soon-Jin;Yoon, Chun-Gyeong
    • Korean Journal of Ecology and Environment
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    • v.35 no.1 s.97
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    • pp.28-35
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    • 2002
  • The effect of physical parameters on water quality was analyzed using monitoring data of 193 agricultural reservoirs. The retention time of reservoirs ($t_d$) ranged between 10 and 140 days, and the ratio of drainage area (DA) to reservoir surface area (SA) was between 10 and 120. Both ratios of DA/SA and total area (TA)/ reservoir storage (ST) in Korean agricultural reservoirs were relatively greater than those in natural lakes in other countries. As retention time was plotted against DA/SA ratio, it was shorter in Korean reservoirs than natural lakes. The semi-logarithmic relationship between TA/SA and t>$t_d$ was $t_d\;=\;42.21(TA/ST)^{-1}$ (n = 50, $R^2\;=\;0.89$). While areal loading of total phosphorus (TP) was below $4\;gTP{\cdot}m^{-2}{\cdot}yr^{-1}$ in general, it exceeded $10\;gTP{\cdot}m^{-2}{\cdot}yr^{-1}$ in reservoirs where DA/SA ratio was greater than 100, which implies that areal loading of TP increases as DA/SA ratio increases. Chl-a concentration was positively related with the mean depth of reservoir, implying the higher Chl-a concentration with deeper the mean depth. Therefore, the deeper reservoir might be advantageous in water quality management perspective if other morphological conditions are similar. The empirical regression equation using physical parameters was also suggested in the estimation of TP concentration in the reservoirs. Combined information presented in this paper might be applicable to the water quality management in agricultural reservoirs.

Purification and Biological Characterization of Wild-type and Mutants of a Levan Fructotransferase from Microbacterium sp. AL-210 (Microbacterium sp. A-210이 생성하는 Levan fructotransferase의 정제 및 생물학적 특성에 관한 연구)

  • Hwang, Eun-Young;Jeong, Mi-Suk;Cha, Jae-Ho;Jang, Se-Bok
    • Journal of Life Science
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    • v.19 no.9
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    • pp.1218-1225
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    • 2009
  • Difractose anhydrides (DFAs) is studied as a sweetener for diabetics because of its structural property. DFAs have four types: DFA I, III, IV (degradation of levan) and V (degradation of inulin). Especially, DFA IV has been shown to enhance the absorption of calcium in experiments using rats. Levan fructotransferase is an enzyme for producing di-d-fructose-2,6':6,2-dianhydride (DFA IV). To identify structural characterization, we purified wild-type and mutants (D63A, D195N and N85S) of levan fructotransferase (LFTase) from Microbacterium sp. AL-210. These proteins were purified to apparent homogeneity by Ni-NTA affinity column, Q-sepharose ion exchange and gel filtration chromatography and detected by SDS-PAGE. They were also analyzed by circular dichroism (CD) measurements, JNET secondary structure prediction, activity measurements at various temperatures, and pH analysis. The optimum pH for the enzyme-catalyzed reaction was pH 7.5 and optimum temperature was observed at $55^{\circ}C$. Along with wild-type LFTase, mutants were analyzed by CD measurement, fluorescence analysis and differential scanning calorimetry (DSC). N85S showed less $\alpha$-helix and more $\beta$ strand than others. Also, N85S showed almost the same curve as wild-type in their steady-state fluorescence spectra, whereas mutant D63A and D195N showed higher intensity than wild-type. The amino acid sequence of wild-type LFTase was compared to the sequences of exo-inulinase from Aspergillus awamori, a plant fructan 1-exohydrolase from Cichorium intybus, and Thermotogo maritime (Tm) invertase and showed a high identity with Exo-inulinase from Aspergillus awamori.

A Study on the Production of Artificial Seed and Intermediate culture for Attached Spats of the Chinese Stock of a Scallop, Patinopecten yessoensis (중국산 참가리비, Patinopecten yessoensis의 인공종묘 생산 및 부착치패 중간양성에 관한 연구)

  • Oh, Bong-Se;Lee, Jeong-Yong;Park, Se-Ku;Lee, Chu;Jo, Q-Tae
    • The Korean Journal of Malacology
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    • v.24 no.2
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    • pp.153-159
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    • 2008
  • We investigated artificial mass seed production of a Chinese scallop, Patinopecten yessoensis, in 2004. The GSI(gonad somatic index) of the Chinese scallop, P yessoensis was 17.2 on mid-February, 20.2 on mid-March, while that of Korean scallop, P yessoensis was 6.9 on mid-February, 10.8 on mid-March. Matured 120 females and 350 males were selected for artificial mass production. They were exposed in air for 1 hr at over $20^{\circ}C$, and placed into a spawning tank(20 ton) containing sea water treated with UV radiation at $12^{\circ}C$. We gained a total of 228,000 thousand scallop embryos between March 10th and 15th, and reared larvae at the indoor tank during 25 days. When the mean shell length of larvae reached 250 ${\mu}m$ and they have eye-spots, the number of pre-settling larvae was 47,500 thousand. We gained 1,850 thousand attached scallop spats from two kinds of collectors. Attached spats were reared in indoor tank for different periods from 5 days to 60 days. They were divided into 5 groups according to the length of reared days. Each group of attached spats was moved to intermediate rearing sites at Yangyang fishing port in Gangreung-city for acclimation to ocean environments. The highest survival rate of attached spats was 13.0% shown at the group reared for 12 days, but the significant difference in their growth was not found between the groups. The shell length of artificial attached spats increased from 0.9 ${\mu}m$ on July 10th to 24.7 ${\mu}m$ on December 16th with the survival rate of 85.0% while that of natural attached spats increased from 0.6 ${\mu}m$ on July 10th to 23.9 ${\mu}m$ on December 16th with the survival rate of 85.7%.

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Variation of Cadmium and Zinc Content in Rice and Soil of the Mangyeong River Area (만경강 유역의 토양과 수도체중 Cd 및 Zn 함량의 변화)

  • Kim, Seong-Jo;Baek, Seung-Hwa;Kim, Un-Sung;Yoon, Ki-Woun;Moon, Kwang-Hyun;Kang, Gyeong-Won
    • Korean Journal of Environmental Agriculture
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    • v.13 no.2
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    • pp.142-150
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    • 1994
  • To investigate differences in Cd and Zn contents of paddy soils and rice plants polluted by the municipal and industrial waste water in the Mangyeong River Area, soil and plant samples were collected at several distances from the main inlet and at different depths of the soil. Soil samples were extracted with $4M-HNO_3$ and plant samples were digested with a mixture of $HNO_3$and $HClO_4$for analyzing heavy metals by atomic absorption spectrophotometry. The contents of Cd and Zn in soils ranged from 0.38 to 1.17 and from 33.8 to 464.6mg kg^{-1}, respectively. The average Cd level in 1990 was less than that in 1982, but the Zn level in 1990 was higher than that in 1982 in general. No variation in Cd contents was observed in soils at the different distances from the source of waste water, but Zn contents in soils were lower with the increasing distances from the source of waste water. A significant correlation was observed among Cd content, OM, available silicate, CEC and $Ca^{++}$. Similar results existed among Zn content of 1982, OM and $Ca^{++}$. The Cd content in subsurface soils of 1992 was significantly correlated with Zn, Cu, and Pb in soils, and the Zn content in soils was significantly correlated with the Cu and Pb in soils, regardless of years. The Cd content in leaf blades of rice was more than seven times higher than that in brown rice. The Zn content in rice was higher than that in leaf blades and in panicle axis. The Cd content in panicle axis and the Zn content in all parts of rice were correlated with Zn, Cu and Pb contents in soils. The Cd and Zn contents in brown rice ranged from 0.10 to 0.90mg $kg^{-1}$ and from 4.2 to 95.9mg $kg^{-1}$ in the Mangyeong River Area, respectively.

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Effects of Boliing, Steaming, and Chemical Treatment on Solid Wood Bending of Quercus acutissima Carr. and Pinus densiflora S. et. Z. (자비(煮沸), 증자(蒸煮) 및 약제처리(藥劑處理)가 상수리나무와 소나무의 휨가공성(加工性)에 미치는 영향(影響))

  • So, Won-Tek
    • Journal of the Korean Wood Science and Technology
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    • v.13 no.1
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    • pp.19-62
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    • 1985
  • This study was performed to investigate: (i) the bending processing properties of silk worm oak (Quercus acutissima Carr.) and Korean red pine (Pinus densiflora S. et Z.) by boiling and steaming treatments; (ii) the effects of interrelated factors - sapwood and heartwood, annual ring placement, softening temperature and time, moisture content. and wood defects on bending processing properties; (iii) the changing rates of bending radii after release from a tension strap, and (iv) the improving methods of bending process by treatment with chemicals. The size of specimens tested was $15{\times}15{\times}350mm$ for boiling and steaming treatments and $5{\times}10{\times}200mm$ for treatments with chemicals. The specimens were green for boiling treatments and dried to 15 percent for steaming treatments. The specimens for treatments with chemicals were soaked in saturated urea solution, 35 percent formaldehyde solution, 25 percent polyethylene glycol -400 solution, and 25 percent ammonium hydroxide solution for 5 days and immediately followed the bending process, respectively. The results obtained were as follows: 1. The internal temperature of silk worm oak and Korean red pine by boiling and steaming time was raised slowly to $30^{\circ}C$ but rapidly from $30^{\circ}C$ to $80-90^{\circ}C$ and then slowly from $80-90^{\circ}C$ to $100^{\circ}C$. 2. The softening time required to the final temperature was directly proportional to the thickness of specimen. The time required from $25^{\circ}C$ to $100^{\circ}C$ for 15mm-squared specimen was 9.6-11.2 minutes in silk worm oak and 7.6-8.1 minutes in Korean red pine. 3. The moisture content (M.C.) of specimen by steaming time was increased rapidly first 4 minutes in the both species, and moderately from 4 to 20 minutes and then slowly and constantly in silk worm oak, and moderately from 4 to 15 minutes and then slowly and constantly in Korean red pine. The M.C. of 15mm-squared specimen in 50 minutes of steaming was increased to 18.0 percent in the oak and 22.4 percent in the pine from the initial conditioned M.C. of 15 percent The rate of moisture adsorption measured was therefore faster in the pine than in the oak. 4. The mechanical properties of the both species were decreased significantly with the increase of boiling rime. The decrement by the boiling treatment for 60 minutes was measured to 36.6-45.0 percent in compressive strength, 12.5-17.5 percent in tensile strength, 31.6-40.9 percent in modulus of rupture, and 23.3-34.6 percent in modulus of elasticity. 5. The minimum bending radius (M.B.R.) of sapwood and heartwood was 60-80 mm and 90 mm in silk worm oak, and 260 - 300 mm and 280 - 300 mm in Korean red pine, respectively. Therefore, the both species showed better bending processing properties in sapwood than in heartwood. 6. The M.B.R. of edge-grained and flat-grained specimen in suk worm oak was 60-80 mm, but the M.B.R. in Korean red pine was 240-280 mm and 260-360 mm, respectively. Comparing the M.B.R. of edge-grained with flat-grained specimen, in the pine the edge-grained showed better bending processing property than the flat-grained. 7. The bending processing properties of the both species were improved by the rising of softening temperature from $40^{\circ}C$ to $100^{\circ}C$. The minimum softening temperature for bending was $90^{\circ}C$ in silk worm oak and $80^{\circ}C$ in Korean red pine, and the dependency of softening temperature for bending was therefore higher in the oak than in the pine. 8. The bending processing properties of the both species were improved by the increase of softening time as well as temperature, but even after the internal temperature of specimen reaching to the final temperature, somewhat prolonged softening was required to obtain the best plastic conditions. The minimum softening time for bending of 15 mm-squared silk worm oak and Korean red pine specimen was 15 and 10 minutes in the boiling treatment, and 30 and 20 minutes in the steaming treatment, respectively. 9. The optimum M.C. for bending of silk worm oak was 20 percent, and the M.C. above fiber saturation point rather degraded the bending processing property, whereas the optimum M.C. of Korean red pine needed to be above 30 percent. 10. The bending works in the optimum conditions obtained as seen in Table 24 showed that the M.B.R. of silk worm oak and Korean red pine was 80 mm and 240 mm in the boiling treatment, and 50 mm and 280 mm in the steaming treatment, respectively. Therefore, the bending processing property of the oak was better in the steaming than in the boiling treatment, but that of the pine better in the boiling than in the steaming treatment. 11. In the bending without a tension strap, the radio r/t of the minimum bending radius t to the thickness t of silk worm oak and Korean red pine specimen amounted to 16.0 and 21.3 in the boiling treatment, and 17.3 and 24.0 in the steaming treatment, respectively. But in the bending with a tension strap, the r/t of the oak and the pine specimen decreased to 5.3 and 16.0 in t he boiling treatment, and 3.3 and 18.7 in the steaming treatment, respectively. Therefore, the bending processing properties of the both species were significantly improved by the strap. 12. The effect of pin knot on the degradation of bending processing property was very severe in silk worm oak by side, e.g. 90 percent of the oak specimens with pin knot on the concave side were ruptured when bent to a 100 mm radius but only 10 percent of the other specimens with pin knot on the convex side were ruptured. 13. The changing rate in the bending radius of specimen bent to a 300 mm radius after 30 days of exposure to room temperature conditions was measured to 4.0-10.3 percent in the boiling treatment and 13,0-15.0 percent in the steaming treatment. Therefore, the degree of spring back after release was higher in the steaming than in the boiling treatment. And the changing rate of moisture-proofing treated specimen by expoxy resin coating was only -1.0.0 percent. 14. Formaldehyde, 35 percent solution, and 25 percent polyethylene glycol-400 solution found no effect on the plasticization of the both species, but saturated urea solution and 25 percent ammonium hydroxide solution found significant effect in comparison to non-treated specimen. But the effect of the treatment with chemicals alone was inferior to that of the steaming treatment, and the steaming treatment after the treatment with chemicals improved 10-24 percent over the bending processing property of steam-bent specimen. 15. Three plasticity coefficients - load-strain coefficient, strain coefficient, and energy coefficient - were evaluated to be appropriate for the index of bending processing property because the coefficients had highly significant correlation with the bending radius. The fitness of the coefficients as the index was good at load-strain coefficient, energy coefficient, and strain coefficient, in order.

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The Effect of Deodeok Contents on the Quality of Deodeok Wine (더덕 함량이 더덕 침출주의 품질에 미치는 영향)

  • Kwon, Dong-Jin;Choi, Shin-Yang
    • Food Science and Preservation
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    • v.14 no.4
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    • pp.414-418
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    • 2007
  • The physicochemical characteristics and sensory properties of Deodeok wine, formed by leaching of Deodeok at room temperature for 180 days, were investigated over the following range of Deodeok levels: 10, 15 and 20% (all w/v). The higher the level of Deodeok, the greater were the final values of total sugars, reducing sugars, total polyphenols, and crude saponins. The Hunter's b-value (yellowness) of Deodeok wine varied markedly with Deodeok levels, and yellowness was highest in Deodeok wine containing 20% (w/v) Deodeok. Non-volatile compounds, that form the basis of the liquor tax law, were 0.64, 1.38 and 2.11% (all w/v), respectively, at day 160. Of these values, that of 2.11% (w/v), the level of non-volatile compounds in Deodeok wine containing 20% (w/v) Deodeok, was in accord with the liquor tax law (that requires this figure to be 2.0%). Sensory evaluation showed that Deodeok wine containing 20% (w/v) Deodeok was superior to the other wines tested.