• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.40-43
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• 2015

Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is an autosomal recessive mitochondrial disorder of fatty acid oxidation associated with mutations in the ACADS gene. While patients diagnosed clinically have a variable clinical presentation, patients diagnosed by newborn screening are largely asymptomatic. We describe here the case of a 1-year-old male patient who was detected by newborn screening and diagnosed as SCAD deficiency. Spectrometric screening for inborn errors of metabolism at 72hrs after birth showed elevated butyrylcarnitine (C4) level of 1.69 mol/L (normal, <0.83 mol/L), C4/C2 ration of 0.26 (normal, <0.09), C5DC+C60H level of 39 mol/L (normal, <0.28 mol/L), and C5DC/C8 ration of 7.36 (normal, <4.45). The follow-up testing at 18 days of age were performed: liquid chromatography tandem mass spectrometry (LC-MS/MS), urine organic acids, and quantitative acylcarnitine profile. C4 carnitine was elevated as 0.91; urine organic acid analysis showed elevated ethylmalonic acid as 62.87 nmol/molCr (normal, <6.5), methylsuccinate 6.81 nmol/molCr (normal, not detected). Sequence analysis of ACADS revealed a homozygous missense mutation, c.164C>T (p.Pro55Leu). He is growing well and no episodes of seizures or growth retardation had occurred.

• Korean Journal of Organic Agriculture
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• v.29 no.4
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• pp.613-623
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• 2021

This study was conducted to investigate the effect of addition levels of coffee and green tea by products extract including polyphenols through hot water extraction on rumen fermentation. The treatment groups consisted of coffee extract (CO), green tea extract (GR) and mixed extract (MIX), and the addition level was 10 µL, 20 µL and 30 µL of three levels. The experiment consisted of a total of 10 experimental groups including the control group, and a full factorial design was used. The effect of polyphenol addition in coffee and green tea by-products was analyzed through main and interaction effect of statistical analysis. The total polyphenol content of the extracts was 106.15, 79.10 and 185.25 ㎍ GAE/g DM for coffee by-product, green tea by-product and mixture, respectively. Total gas production was significantly lower in the treatment groups than in the control (114.00 mL/gDM) (p<0.05). Methane emission tended to decrease as the polyphenol addition level increased. Moreover, the MIX showed the lowest methane emission when 30 µL was added (p<0.05). Volatile fatty acids showed a significant difference compared to the treatment group as a control (98.06 mM) (p<0.05), but there was no change according to the level of polyphenols. As a result of the main effect and interaction, it is thought that the effect on methane reduction and improvement of rumen fermentation in MIX20 can be expected. In a series of studies, the addition of 20 µL of a blended extract of coffee and green tea by-products is thought to reduce methane to levels that do not inhibit rumen fermentation.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.199-204
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• 2009

Purpose : Seizure associated with fever may indicate the presence of underlying inherited metabolic diseases. The present study was performed to investigate the presence of underlying metabolic diseases in patients with complex febrile seizures, using analyses of urine organic acids. Method : We retrospectively analyzed and compared the results of urine organic acid analysis with routine laboratory findings in 278 patients referred for complex febrile seizure. Results : Of 278 patients, 132 had no abnormal laboratory findings, and 146 patients had at least one of the following abnormal laboratory findings: acidosis (n=58), hyperammonemia (n=55), hypoglycemia (n=21), ketosis (n=12). Twenty-six (19.7 %) of the 132 patients with no abnormal findings and 104 (71.2%) of the 146 patients with statistically significant abnormalities showed abnormalities on the organic acid analysis (P<0.05). Mitochondrial respiratory chain disorders (n=23) were the most common diseases found in the normal routine laboratory group, followed by PDH deficiency (n=2) and ketolytic defect (n=1). In the abnormal routine laboratory group, mitochondrial respiratory chain disorder (n=29) was the most common disease, followed by ketolytic defects (n=27), PDH deficiency (n=9), glutaric aciduria type II (n=9), 3-methylglutaconic aciduria type III (n=6), biotinidase deficiency (n=5), propionic acidemia (n=4), methylmalonic acidemia (n=2), 3-hydroxyisobutyric aciduria (n=2), orotic aciduria (n=2), fatty acid oxidation disorders (n=2), 2-methylbranched chain acyl CoA dehydrogenase deficiency (n=2), 3-methylglutaconic aciduria type I (n=1), maple syrup urine disease (n=1), isovaleric acidemia (n=1), HMG-CoA lyase deficiency (n=1), L-2-hydroxyglutaric aciduria (n=1), and pyruvate carboxylase deficiency (n=1). Conclusion : These findings suggest that urine organic acid analysis should be performed in all patients with complex febrile seizure and other risk factors for early detection of inherited metabolic diseases.

• Food Science and Preservation
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• v.13 no.2
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• pp.186-191
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• 2006

Prunus mume is extensively cultivated as a fruit and medicinal plant in Korea. Recently, prunus mume has a pressing problem with an increase of prunus mume cultivation area in southern part in Korea. Chemical properties of prunus mume flower to determine the optimum processing varieties for tea were investigated. Three kinds of samples treated with fresh, freeze dry and shade dry were used. The content of moisture, crude ash, crude protein, crude fiber, crude fat and nitrogen free extract of prunus mume flower varieties were to $82{\sim}85%,\;0.2{\sim}0.6%,\;2.5{\sim}3.1%,\;2.5{\sim}3.1%,\;0.6{\sim}0.8%\;and\;10{\sim}11%$ respectively. The main component of free sugars in prunus mume flower was glucose and those of organic acids were citric and malic acids. 17 kinds of amino acids were determined from prunus mume flower. The total amino acid contents of Cheongchuk, Baeagaha and Goseong were 760.47 mg%, 624.01 mg% and 807.41 mg%, respectively. Aspartic acid, glutamic acid and lysine were the major component in 3 cultivars. The content of K was much higher than Ca, Mg, Na, fe and Zn. The major fatty acids of prunus mume flower were myristic acid, palmitoleic acid me oleic acid. As a result of analysis, there were no significant differences among the three cultivars of prunus mume flower and drying method.

• Journal of the Korea Organic Resources Recycling Association
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• v.25 no.3
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• pp.91-98
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• 2017

The purpose of this study is to provide a design and operation technical guideline for meeting the appropriate design criteria to bio-gasification facilities treating organic wastes. 9 anaerobic digestion facilities which is normally operated during the field survey and 14 livestock manure farms were selected for precision investigation. the physicochemical analysis was performed on the moisture and organic contents, nutrients composition (carbohydrate, fat, protein), volatile fatty acids (VFAs), and nitrogen, etc. Volatile solids (VS) of organic wastes brought into the bio-gasification facilities were 2.81 % (animal manure only) and 5.92 % (animal manure+food waste), respectively. Total solids (TS) reults of samples from livestock farms were 5.6 % in piglets and 11~13 % in other kinds of breeding pigs. The actual methane yield based on nutrients contents was estimated to $0.36Sm^3CH_4/kgVS$ which is equivalent to 72 % of theoretical methane yield value. The optimum mixing ratio depending on the effect of the combined bio-gasification was obtained through the continuous stirred-tank reactor (CSTR) which is operated at different mixing ratio of swine manure and food waste leachate. The range of swine manure and food waste leachate from 60:40 to 40:60 were adequate to the appropriate conditions of anaerobic digestion; less than 100 gTS/, more than alkalinity of 1 gCaCO3/L, C/N ratio 12.0~30.0, etc.

• Korean Journal of Food Science and Technology
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• v.31 no.4
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• pp.912-918
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• 1999

The chemical compositions of korean and Chinese safflower (Carthamus tinctorious L.) seed were compared in this study. The proximate compositions were 0.73 and 0.05% of moisture, 19.74 and 18.82% of crude protein, 15.47 and 14.61% of crude fat, 3.78 and 3.87% of crude ash, 14.53 and 10.46% of crude fiber, 46.49 and 52.23% of N-free extracts in the non-roasted safflower seed (NRS) and roasted safflower seed (RS), respectively. Crude fat contents in non-roasted chinese safflower seed (NRCS) and roasted chinese safflower seed (RCS) were 33.30 and 31.22%, which were higher than those of NRS and RS. Unsaturated fatty acid in NRS was 83.2% and 90.9% in NRCS. Linoleic acid was the most predominant fatty acid in NRS (74.0%) and NRCS (74.2%). Sucrose (216.5 mg/100 g) and raffinose (117.5 mg/100 g) were major free sugars in NRS, but sucrose, glucose, fructose and raffinose were in NRCS. Glutamic acid, aspatic acid and arginine were major in total amino acids. 24 kinds of free amino acid were detected in NRS and 11 kinds in RS. Total essential amino acid in NRS ($28.0\;{\mu}g/100\;g$) was higher than that in NRCS ($9.2\;{\mu}g/100\;g$). The organic acids in safflower seed were composed of formic acid, succinic acid, malic acid, oxalic acid and fumaric acid. The content of vitamin E $({\alpha}-tocopherol)$ in NRS and NRCS were 10.5 mg/100 g, 6.2 mg/100 g, NRCS and RCS were 12.8 mg/100 g, 9.4 mg/100 g, respectively. Total carotenoid content in NRCS was $452.0\;{\mu}g/100\;g$ and it was higher than in NRS. The major minerals of safflower seed were K, P, Ca, Mg.

• Journal of Life Science
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• v.23 no.3
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• pp.415-425
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• 2013

This study compared the characteristics of five Bacillus strains capable of aerobic and anaerobic growth, CBW3, CBW4, CBW9, CBW14 and EBW10. They were isolated and selected from a polychaete, Perinereis aibuhitensis, which is known as a good degrader of organic compounds in marine wetland. Based on a 16S rRNA sequence, CBW3 and CBW14 were found to share more than 99.8% similarity with B. nanhaiensis, B. arsenicus and B. barbaricus. CBW4, CBW9 and EBW10 shared 92.7%, 99.8%, and 99.8% similarity with B. anthracis, B. algicoa and B. thuringiensis, respectively. The temperature, salinity, and pH ranges of the cell growth of the Bacillus strains were $4-45^{\circ}C$, 0-17%, and pH 5-pH 9, respectively. All Bacillus strains were found to exhibit enzyme activities for the degradation of casein and starch. Notably, strain EBW10 exhibited the enzyme activities for all the tested macromolecules, DNA, casein, starch, cellulose, and four kinds of Tweens, which suggests the possibility that it had protease, amylase, cellulose, and lipase. All five Bacillus strains had alkaline phosphatase activities, and the strains CBW3, CBW4, and EBW10 also had acid phospatase. Strains CBW3 and EBW10 exhibited the enzyme activities both for esterase (C4) and esterase lipase (C8). The analysis of fatty acids revealed that in all strains, major fatty acids were anteiso $C_{15:0}$ and iso $C_{15:0}$.

• Korean Journal of Plant Resources
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• v.31 no.4
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• pp.283-293
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• 2018

This study was performed to analysis of chemical constituent in Polygonum multiflorum root (PMR) by different dry methods (hot-air dry, shade dry, and freeze dry). The results are summarized as followings; major free sugar were detected fructose, glucose, and sucrose in dried PMR based on various dry methods. The highest content of free sugars was found in freeze dried PMR. The four organic acids were detected in dried PMR by HPLC analysis. The content of oxalic acid in shade dried PMR was higher than the dried PMR by different dry methods. The content of total amino acid and essential amino acids were high in the orders of freeze drying > shade drying > hot-air drying. The potassium and magnesium levels of freeze dried PMR was significantly higher than the other drying method of PMR. Whereas the calcium and sodium levels were higher in hot-air dried PMR. The major fatty acids were determined the linoleic acid in PMR by different dry methods.

• Asian-Australasian Journal of Animal Sciences
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• v.18 no.12
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• pp.1715-1720
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• 2005

Two promising varieties of forage sorghum viz. HD-15 and J. Sel-10 were identified for their higher forage yield (350-400 quintals per hectare) compared to traditional/existing variety, HC-136 at Indian Grassland and Fodder Research Institute, Jhansi. Silage of these three forage sorghum varieties viz., HD-15, J.Sel-10 and HC-136 were prepared in three concrete silos for their nutritional evaluation in sheep. Twelve adult Muzaffarnagari Ewes, divided into three equal groups of 4 each, were offered a particular variety of sorghum silage ad libitum over a period of 30 days followed by a 6 day metabolism trial and a collection of rumen liquor and blood samples. The HD-15 variety of forage sorghum silage contained higher crude protein (CP) than both the J.Sel-10 and HC-136. Average daily dry matter (DM) intake (% of live weight) differed significantly (p<0.05) in sheep that were fed the different varieties of silage, with the maximum amount in HD-15 (2.55) followed by J.Sel-10 (2.49) and HC-136 (1.84). The average apparent digestibility of all the nutrients was low in the sheep that were fed the HC-136 variety of sorghum silage when compared to both HD-15 and J.Sel-10. However, digestibility of organic matter (p<0.05) and crude protein (p<0.01) was significantly low in the HC-136 variety. The average daily nitrogen retention was-0.19, 2.15 and 0.42 g in HC-136, HD-15 and J.Sel-10, respectively which differed significantly (p<0.01). The average digestible crude protein and total digestible nutrient (TDN) contents (%) of the silage varieties were higher in HD-15 (3.14 and 55.3) than HC-136 (0.25 and 58.6) and J.Sel-10 (1.58 and 55.3) varieties. On comparing to the maintenance requirements (ICAR, 1985) for dry matter, crude protein and total digestible nutrients, both the improved varieties (HD-15 and J.Sel-10) almost met the requirements except crude protein, which met only 73.3% of the requirement by J.Sel-10, while the HC-136 variety was unable to meet the requirements. The average rumen pH and total volatile fatty acids concentrations were comparable among the groups. However, concentrations of nitrogen metabolites were higher in the animals fed the HD-15 variety of silage. Varieties of sorghum silage also did not have any influence on the concentration of the blood metabolites. It was concluded that the HD-15 variety of sorghum silage was nutritionally superior to both J.Sel-10 and HC-136 silage.

• Asian-Australasian Journal of Animal Sciences
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• v.27 no.11
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• pp.1562-1570
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• 2014

The effects of Lactobacillus mucosae (L. mucosae), a potential direct fed microbial previously isolated from the rumen of Korean native goat, on the rumen fermentation profile of brewers grain were evaluated. Fermentation was conducted in serum bottles each containing 1% dry matter (DM) of the test substrate and either no L. mucosae (control), 1% 24 h broth culture of L. mucosae (T1), or 1% inoculation with the cell-free culture supernatant (T2). Each serum bottle was filled anaerobically with 100 mL of buffered rumen fluid and sealed prior to incubation for 0, 6, 12, 24, and 48 h from which fermentation parameters were monitored and the microbial diversity was evaluated. The results revealed that T1 had higher total gas production (65.00 mL) than the control (61.33 mL) and T2 (62.00 mL) (p<0.05) at 48 h. Consequently, T1 had significantly lower pH values (p<0.05) than the other groups at 48 h. Ammonia nitrogen ($NH_3$-N), individual and total volatile fatty acids (VFA) concentration and acetate:propionate ratio were higher in T1 and T2 than the control, but T1 and T2 were comparable for these parameters. Total methane ($CH_4$) production and carbon dioxide ($CO_2$) were highest in T1. The percent DM and organic matter digestibilities were comparable between all groups at all times of incubation. The total bacterial population was significantly higher in T1 (p<0.05) at 24 h, but then decreased to levels comparable to the control and T2 at 48 h. The denaturing gradient gel electrophoresis profile of the total bacterial 16s rRNA showed higher similarity between T1 and T2 at 24 h and between the control and T1 at 48 h. Overall, these results suggest that addition of L. mucosae and cell-free supernatant during the in vitro fermentation of dried brewers grain increases the VFA production, but has no effect on digestibility. The addition of L. mucosae can also increase the total bacterial population, but has no significant effect on the total microbial diversity. However, inoculation of the bacterium may increase $CH_4$ and $CO_2$ in vitro.