Sook Za Kim;Wung Joo Song;Sun Ho Lee;Harvey L. Levy
Journal of The Korean Society of Inherited Metabolic disease
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v.23
no.2
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pp.31-38
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2023
Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder caused by a deficiency in branched chain α-keto acid dehydrogenase (BCKAD). Between 1997, when Korea's MSUD case was first reported, and 2023, 14 cases were reported in the literature. 29% of the cases experienced developmental delay, and 29% expired. The prevalence of MSUD in Korea was estimated to be 1 in 230,000. Of 21 MSUD patients currently being treated at the Korea Genetics Research Center, 19 were detected through newborn screening program, and 2 were diagnosed by the symptoms. 14 MSUD patients had confirmed genetic mutations; 6 (43%) were BCKDHA and 8 (57%) were BCKDHB. In one case, a large deletion was observed. 4 patients had leucine levels above 2,000 (umo/L), and post-dialysis diet therapy was initiated in the newborn period. No patient required further dialysis as diet therapy and regular monitoring proved highly effective. Most MSUD patients were growing normally; weight and height growth were above the 50th percentile in 76% of the cases while BMI values were higher than normal in 71% of cases. Developmental delays were observed only in 2 cases (10%) and anticonvulsant use in 3 cases (14%). With newborn screening available to all Korean infants, early diagnosis and intervention should allow most patients to remain asymptomatic. However, ongoing surveillance, dietary management and continued patient compliance as well as rapid correction of acute metabolic decompensations remain critical to a favorable long-term prognosis.
The study was conducted to find out the gene effects on body weights at weaning and at the age of 60 days in mice, with 343 progenies from full-dialell crosses of four lines of BALB/c, CBA, C3H and C57BL. The progenies were reared at the Experimental Animal Farm, College of Agriculture, Kyungpook National University from November, 1984 to February, 1985, and data collected from the progenies were analyzed into general combining ability, maternal effects, specific combining ability and reciprocal effects with Harvey's model. General combining ability effects estimated in line-crosses were -0.6033~0.5298 for weaning weights and -0.5086~1.0012 for body weights at the age of 60 days. General combining ability for BALB/c and C57BL were significantly better than general combining ability for CBA and C3H for both traits (P<0.05). Maternal effects for C3H were significantly larger than the maternal effects of BALB/c for both traits (P<0.05). The estimates of maternal effects were -0.9678~0.4609 for weaning weights and -1.1886~0.0729 for body weights at the age of 60 days. Specific combining ability effects were estimated to be significant (P<0.05), and the estimates were -0.1999~0.3380 for weaning weights and -0.4056~0.3317 for body weights at the age of 60 days. Reciprocal effects were found to be largest in BALB/c${\times}$C57BL and BALB/c${\times}$C3H. The estimates were -0.5049 from BALB/c${\times}$C57BL and 0.4972 from BALB/c${\times}$C3H form weaning weights, and -1.0336 from BALB/c${\times}$C57BL and 1.2793 from BALB/c${\times}$C3H for body weights at the age of 60 days.
Journal of the Korean Applied Science and Technology
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v.35
no.4
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pp.1487-1495
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2018
In this study, we investigated collagen production and hyaluronic acid production effects for wrinkle improvement test on 50 kinds of land plants and 10 kinds of marine plants native to Jeju Island as a part of developing customized cosmetic materials. Collagen and hyaluronic acid are recognized as major factors affecting skin aging. Cerastium holosteoides var. hallaisanense Mizushima extract ($100{\mu}g/mL$) produced more than 190% of collagen in the extracts of 50 kinds of land plants. Vicia angustifolia var. segetilis K. Koch. extract ($100{\mu}g/mL$) produced more than 160% of collagen. Ftsia japonica Decne. et Planch. extract ($100{\mu}g/mL$), Euonymus japonica Thunb. extract ($100{\mu}g/mL$), Suaeda malacosperma H.Hara extract ($100{\mu}g/mL$), Elaeagnus umbelellata Thunb. extract ($100{\mu}g/mL$), Sedum oryzifolium Makino extract ($100{\mu}g/mL$), Vicia unijuga A. Br. extract ($100{\mu}g/mL$), and Brassica juncea var. integrifolia Sinsk. extract ($100{\mu}g/mL$) showed more than 140% collagen production effect. Among the 10 species of marine plants, Sargassum macrocarpum C. Agardh extract ($50{\mu}g/mL$) produced more than 190% of collagen, and Carpopeltis angusta (Harvey) Okamura extract ($100{\mu}g/mL$), Codiumcoactum Okamura extract ($100{\mu}g/mL$), and Codium tenuifolium S. Shimada, T. Tadano & J. Tanaka extract ($100{\mu}g/mL$) showed more than 140% collagen production. Suaeda malacosperma H.Hara extract ($100{\mu}g/mL$) showed the effect of producing hyaluronic acid more than 140%, and Ftsia japonica Decne. et Planch. extract ($20{\mu}g/mL$) and Wistaria floribunda A.P. DC extract ($100{\mu}g/mL$) showed more than 130% hyalunonic acid production effect. Among the 10 species of marine plants, Peyssonnelia capensis Montagne extract ($100{\mu}g/mL$) was the most effective. Carpopeltis angusta (Harvey) Okamura extract ($100{\mu}g/mL$), Codiumcoactum Okamura extract ($100{\mu}g/mL$), and Codium tenuifolium S. Shimada, T. Tadano & J. Tanaka extract ($100{\mu}g/mL$) showed more than 120% hyalunonic acid production. Jeju resources, which have good collagen and hyaluronic acid production, showed the potential to be applied to solve the skin troubles of customized cosmetics in the future.
Journal of the Korean Society of Food Science and Nutrition
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v.31
no.1
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pp.87-91
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2002
This study was designed to investigate the effects of Hijikia fusiforme (Harvey) Okamura ethanol extract on the ethanol-induced hepatotoxicity of rat administered orally experimental diets for 6 weeks. Sprague-Dawley rats weighing about 100 g were divided into 4 groups; normal group (NOR), ethanol (35% ethanol 10 mL/kg b.w/day) treated group (CON), ethanol and Hijikia fusiforme ethanol extract 200 mg/kg (HE1) and 400 mg/kg (HE2) concomitantly treated group, respectively. Each group was examined for the growth rate, feed efficiency ratio (FER), activities of antioxidative enzymes and contents of TBARS and glutathione. Hijikia fusiforme ethanol extract showed increasing effects of the growth rate by 43%, and FER was gradually increased by Hijikia fusiforme ethanol extract treatment, compard with ethanol treatment. Ethanol elevated the activities of superoxide dismutase, catalase and glutathione peroxidase of rat liver markedly as compared to normal group, but those activities were significantly decreased in Hijikia fusiforme ethanol extract treatment by 56%, 38% and 25%, respectively. Xanthine oxidase activity elevated by ethanol was not affected by Hijikia fusiforme ethanol extract. The content of TBARS increased by ethanol treatment was signigicantly decreased in HE2, and the glutathione content depleted by ethanol treatment was increased by Hijikia fusiforme ethanol extract administration adjacent to normal level. These results suggest that Hijikia fusiforme ethanol extract is believed to be a possible protective effect for the ethanol-induced hepatotoxicity of rat liver.
Journal of the Economic Geographical Society of Korea
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v.14
no.3
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pp.263-285
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2011
Despite the process of neoliberalization has made a decisive influence on our society as a whole, there seems little interests in neoliberal urbanization and entrepreneurial urban projects promoting it. This study is to see relationships between neolibealization process and recent urbanization and urban policies in terms of entrepreneurial city mediating them. In particular, this paper tries to reconceptualize entrepreneurial city as corresponding to privatization and commodification, financialization, state redistribution, and management and manipulation of crises which Harvey(2005) suggests as four main features of neoliberalization process in general and 'accumulation by dispossession' in particular, and to characterize it in terms of 'creative destruction' and of 'entrepreneurial governance'. As examined in the later part of this paper, recent cases of these entrepreneurial urban projects in S. Korea include volatility of land and housing price in the Capital region and urban regeneration and newtown projects, project for free economic zones construction in Incheon and other 5 regions, project for SOC construction heavily relying on private investment, in particular project financing pursued nationwidely including Daegu, and project of urban cultural marketing to promote capital inflow tacitly as well as to enhance urban imagine explicitly.
Journal of the Korean association of regional geographers
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v.10
no.1
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pp.34-52
/
2004
This paper aims to examine the construction of the new administrational capital planned by the current government in terms of uneven regional development, and consider important tasks and prospects of the development of the Capital Region. The over-concentration to the Capital region and thereby ever-increasing socio-spatial problems since the 1960s seems to be a crucial aspect of uneven regional development inherent in the process of capitalist development. The construction of the new administrational capital hence can be seen as a version of what Harvey calls 'spatio-temporal fix'. On the other hand, some tasks and prospects of the development of the Capital region can be suggested to become a world city-region. Therefore, it fan be argued that the construction of the new administrational capital would produce its maximum effects, when it will conducted with policies of regional decentralization to overcome or reduce the ongoing uneven geographical development and those of economic and spatial restructuring of the Capital region to resolve or mitigate its socio-spatial problems and lead to a further development as a world city-region as well as a systematic planning of the new administrational capital itself.
The study was conducted to develop the suitable abalone's diets for the indoor tank culture. The young abalone Haliotis discus hannai Ino sized ca. 20, 30 and 40 nun in shell length were fed three different diets, dried sea mustard Undaria pinnatifida HARVEY (control), artificial diet and terrestrial plants e. g. Chinese cabbage and Perilla leaf for 2 years. The growth rate of abalones fed an artificial diet was slightly higher than that of abalones from the control. However, the growth rate of abalones from terrestrial plants was significantly lower than that of abalones fed a control or an artificial diet (P< 0.05). The daily feeding rate of young abalones from terrestrial plants was slightly higher than that of abalones from the control. While, that of abalones from an artificial diet was approximately $30\%$ of that of abalones from a control or a terrestrial plants diet. The feed efficiency of abalone from artificail diet was higher than that of abalones from a control or a terrestrial plants diet, and the smaller size of young abalone showed the higher the feed efficiency. The EFA index values ${{\sum}n9/(20:4n6+20:5n3+22:6n3}$ of the dried sea mustard and the artificial diet were 1.26 and 3.64, respectively. These values were favorable, but the value of terrestrial plant, Perilla leaf was 127.00 indicating poor EFA index for normal growth of abalone.
Objectives: The survival outcomes of antiretroviral treatment (ART) programs have not been systematically evaluated at the state level in India. This retrospective study assessed the survival rates and factors associated with survival among adult human immunodeficiency virus (HIV)-infected patients in Andhra Pradesh, India. Methods: The present study used data from 139 679 HIV patients aged ${\geq}15$ years on ART who were registered from 2007 to 2011 and were followed up through December 2013. The primary end point was death of the patient. Mortality densities (per 1000 person-years) were calculated. Kaplan-Meier and Cox-regression models were used to estimate survival and explore the factors associated with survival. Results: The overall median follow-up time was 16.0 months (2.0 months for the deceased and 14.0 months for those lost to follow-up). Approximately 13.2% of those newly initiated on ART died during follow-up. Of those deaths, 56% occurred in the first three months. The crude mortality rate was 80.9 per 1000 person-years at risk. The CD4 count (adjusted hazard ratio [aHR],4.88; 95% confidence interval [CI], 4.36 to 5.46 for < $100cells/mm^3$ vs. > $350cells/mm^3$), functional status (aHR, 3.05; 95% CI, 2.82 to 3.30 for bedridden vs. normal), and body weight (aHR, 3.69; 95% CI, 3.42 to 3.97 for <45 kg vs. >60 kg) were strongly associated with the survival of HIV patients. Conclusions: The study findings revealed that high mortality was observed within the first three months of ART initiation. Patients with poor baseline clinical characteristics had a higher risk of mortality. Expanded testing and counseling should be encouraged, with the goal of ensuring early enrollment into the program followed by the initiation of ART in HIV-infected patients.
A synthesis of early exploration and the discovery of marine algae in the upper North Pacific and Bering Sea is presented covering the period from the late 1730s to around 1900. Information is provided about these early efforts to gather natural objects, including seaweeds, and names of these algae are enumerated. The first collections of marine algae in this broad region were those made by steller and Kracheninnkov from the Kamchatka Peninsula,Russia,during the Second Kamchatkan Expedition (1735-1742) and were described by Gmelin (1768). The first known algal collections in Alaska were those made byMerck in his 1790-1791 visits to Unalaska Island during the Billings expedition (1785-1794). British-sponsored expeditions for commercial purposes and for exploration and dis-covery allowed surgeon-naturallist Archibald Menzies to garher seaweeds that Dawson Turner and others worked up back in Europe. Several of the Russian Expeditions during the first half of the 18'!' century had naturalists aboard. the first Russian circumnavigation of the globe (1803-1806), with the ships 'Nadeshda' and 'Neva,' under the com-mand of Capt. Adam von Krusenstern had naturalists Langsdorff, Tilesius, and Horner, all of whom collected sea-weeds. The naturalist Adelbert Chanmisso accompanied the Romanzof Expedition (1815-1818) on the Russian vessel 'Rurik' under the command of Otto von Kotzebue and made collections of algae in the Aleutians as well as in the Kurils and Kamchatka. The Lutke expedition of 1826-1829 consisted of thw ships. Feodor Lutke was in command of the 'Seniavin' with K.H. Mertens aboard as physician-naturalist, and the 'Moller' was under the command of staniukovich accompanied by the naturalist G. Kastalsky. The first American-sponsored scientific expedition (1838-1842) was that commanded by Charles Wilkes, and the algae that were collected were worked up by J.W. Bailey and W.H. Harvey. The Russian naturalist Ilya Voznesenskii spent the period 1839-1849 in Russian Americ (Alaska and northern California) energetically traveling and making numerous collections of natural objects as well as ethno-graphic artefact. His algae were described by F.j. Ruprecht back in St. petersbung. The Swedish scientific vessel, the'Vega' (1878-1880), was under the command of Nordenskiold. The naturalist F.R. Kjellman made algal collections from Port Clarence, Alaska, as well as from bering Island and St. Lawrence Island in the Bering sea. The Harriman Alaskan Expedition in the summer of 1899, with the ship 'George W. Elder,' was sponsored by railroad magnate E.H. Harriman of New York City and had several scientific personnel aborad, including the phycologist De Alton Saunders. Algae were collected in Alaska and Washington. During the same summer of 1899 a scientific expedition organized by the University of California and including W.L. Jepson, L.E. Hunt, A.A Lawson, and W.A. Setchell as participants also visited Alaska and made collections of alage from various locations.
Objective: The present study was aimed primarily for estimating various genetic parameters (heritability, genetic correlations) of reproduction (age at first calving [AFC], first service period [FSP]); production (first lactation milk, solid-not fat, and fat yield) and lifetime traits (lifetime milk yield, productive life [PL], herd life [HL]) in Tharparkar cattle to check the association of reproduction traits with lifetime traits through two different methods (Frequentist and Bayesian) for comparative purpose. Methods: Animal breeding data of Tharparkar cattle (n = 964) collected from Livestock farm unit of ICAR-NDRI Karnal for the period 1990 through 2019 were analyzed using a Frequentist least squares maximum likelihood method (LSML; Harvey, 1990) and a multi-trait Bayesian-Gibbs sampler approach (MTGSAM) for genetic correlations estimation of all the traits. Estimated breeding values of sires was obtained by BLUP and Bayesian analysis for the production traits. Results: Heritability estimates of most of the traits were medium to high with the LSML (0.20±0.44 to 0.49±0.71) and Bayesian approach (0.24±0.009 to 0.61±0.017), respectively. However, more reliable estimates were obtained using the Bayesian technique. A higher heritability estimate was obtained for AFC (0.61±0.017) followed by first lactation fat yield, first lactation solid-not fat yield, FSP, first lactation milk yield (FLMY), PL (0.60±0.013, 0.60±0.006, 0.57±0.024, 0.57±0.020, 0.42±0.025); while a lower estimate for HL (0.38±0.034) by MTGSAM approach. Genetic and phenotypic correlations were negative for AFC-PL, AFC-HL, FSP-PL, and FSP-HL (-0.59±0.19, -0.59±0.24, -0.38±0.101 and -0.34±0.076) by the multi-trait Bayesian analysis. Conclusion: Breed and traits of economic importance are important for selection decisions to ensure genetic gain in cattle breeding programs. Favourable genetic and phenotypic correlations of AFC with production and lifetime traits compared to that of FSP indicated better scope of AFC for indirect selection of life-time traits at an early age. This also indicated that the present Tharparkar cattle herd had sufficient genetic diversity through the selection of AFC for the improvement of first lactation production and lifetime traits.
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