• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.2
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• pp.39-44
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• 2023

Hereditary tyrosinemia type 1 (HT-1) is a metabolic disorder caused by biallelic pathogenic variants in the fumarylacetoacetate hydrolase (FAH) gene, which impairs the function of the FAH enzyme, resulting in the accumulation of tyrosine's toxic metabolites in hepatocytes and renal tubular cells. As a consequence, individuals with HT-1 exhibit symptomatic manifestations. Rapid diagnosis and treatment of HT-1 can prevent short-term death and long-term complications. A 15-day-old boy presented to the outpatient department with elevated levels of tyrosine on his newborn screening tests conducted at the age of 3 and 10 days, respectively. Further blood tests revealed increased levels of alpha-fetoprotein and amino acids including tyrosine and threonine. Urine organic acid tests indicated a significant elevation in tyrosine metabolites, as well as the presence of succinylacetone (SA), which led to the diagnosis of HT-1. Two pathogenic and likely pathogenic variants of FAH compatible with HT-1 were also detected. He began a tyrosine-restricted diet at one month old and received nitisinone (NTBC) at two months old. With continued treatment, the patient's initially elevated AFP level, detection of SA in the urine, and mild hepatomegaly showed improvement. During four years and seven months of treatment, there were no exceptional complications apart from an increase in tyrosine levels and a delay in speech. We report a case of tyrosinemia type 1 detected through newborn screening, treated with dietary restriction and NTBC, with a good prognosis.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.26 no.1
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• pp.58-68
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• 2022

Backgrounds/Aims: Mechanisms for the development of hepatocellular carcinoma (HCC) in hepatitis B virus (HBV)-infected patients remain unclear. The aim of the present study was to identify genes and pathways involved in the development of HBV-associated HCC. Methods: The GSE121248 gene dataset, which included 70 HCCs and 37 adjacent liver tissues, was downloaded from the Gene Expression Omnibus database. Differentially expressed genes (DEGs) in HCCs and adjacent liver tissues were identified. Gene ontology and Kyoto Encyclopedia of Genes and Genome pathway enrichment analyses were then performed. Results: Of 134 DEGs identified, 34 were up-regulated and 100 were down-regulated in HCCs. The 34 up-regulated DEGs were mainly involved in nuclear division, organelle fission, spindle and midbody formation, histone kinase activity, and p53 signaling pathway, whereas the 100 down-regulated DEGs were involved in steroid and hormone metabolism, collagen-coated extracellular matrix, oxidoreductase activity, and activity on paired donors, including incorporation or reduction of molecular oxygen, monooxygenase activity, and retinol metabolism. Analyses of protein-protein interaction networks with a high degree of connectivity identified significant modules containing 14 hub genes, including ANLN, ASPM, BUB1B, CCNB1, CDK1, CDKN3, ECT2, HMMR, NEK2, PBK, PRC1, RACGAP1, RRM2, and TOP2A, which were mainly associated with nuclear division, organelle fission, spindle formation, protein serine/threonine kinase activity, p53 signaling pathway, and cell cycle. Conclusions: This study identified key genes and carcinogenic pathways that play essential roles in the development of HBV-associated HCC. This may provide important information for the development of diagnostic and therapeutic targets for HCC.

• Asian-Australasian Journal of Animal Sciences
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• v.33 no.6
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• pp.879-887
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• 2020

Objective: Numerous studies have indicated that the apoptosis and proliferation of granulosa cells (GCs) are closely related to the normal growth and development of follicles and ovaries. Previous evidence has suggested that miR-126-3p might get involved in the apoptosis and proliferation of GCs, and phosphatidylinositol 3-kinase regulatory subunit 2 (PIK3R2) gene has been predicted as one target of miR-126-3p. However, the molecular regulation of miR-126-3p on PIK3R2 and the effects of PIK3R2 on porcine GCs apoptosis and proliferation remain virtually unexplored. Methods: In this study, using porcine GCs as a cellular model, luciferase report assay, mutation and deletion were applied to verify the targeting relationship between miR-126-3p and PIK3R2. Annexin-V/PI staining and 5-ethynyl-2'-deoxyuridine assay were applied to explore the effect of PIK3R2 on GCs apoptosis and proliferation, respectively. Real-time quantitative polymerase chain reaction and Western Blot were applied to explore the regulation of miR-126-3p on PIK3R2 expression. Results: We found that miR-126-3p targeted at PIK3R2 and inhibited its mRNA and protein expression. Knockdown of PIK3R2 significantly inhibited the apoptosis and promoted the proliferation of porcine GCs, and significantly down-regulated the mRNA expression of several key genes of PI3K pathway such as insulin-like growth factor 1 receptor (IGF1R), insulin receptor (INSR), pyruvate dehydrogenase kinase 1 (PDK1), and serine/threonine kinase 1 (AKT1). Conclusion: MiR-126-3p might target and inhibit the mRNA and protein expressions of PIK3R2, thereby inhibiting GC apoptosis and promoting GC proliferation by down-regulating several key genes of the PI3K pathway, IGF1R, INSR, PDK1, and AKT1. These findings would provide great insight into further exploring the molecular regulation of miR-126-3p and PIK3R2 on the functions of GCs during the folliculogenesis in female mammals.

• Journal of Life Science
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• v.14 no.3
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• pp.525-530
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• 2004

This study has been performed to examine physico-chemical properties and composition of venison in Deer breeds. According the results, moisture content in the venison ranged between 74% and 75%, and there were no significant differences among the breeds, or among the cutting parts. Fat content in the meats was estimated as less than 1% except in loin, protein contents was estimated as more than 22%, and ash content was estimated as more than 1.5%. For the contents of inorganic material in the loin, Fe$^{3+}$ content was higher in Elk than in other breeds, the contents of $Cu^{2+}$, $Mn^{2+}$, $K^{+}$, $Mg^{2+}$ and $Ca^{2+}$were higher in Red deer than in others, and the contents of $Fe^{3+}$, $K^{+}$, and $P^{+}$ were higher in Sika deer than in others. For the contents of inorganic material in the leg, the contents of $Cu^{2+}$, $Zn^{2+}$, $K^{+}$, $Ca^{2+}$ and $Mg^{2+}$ were higher in Elk than in other breeds, the contents of $Fe^{3+}$ and$P^{+}$ were higher in Red deer than in others, and the contents of $Cu^{2+}$ and $Fe^{3+}$were higher in Sika deer than in others. Amino acid contents in the venison alanine, proline, glycine, lycine and histidine were highly contained. and the contents of asparagine, cysteine, isoleucine and threonine were low. For the differences of amino acid contents among the breeds, the level was the higher in Sika deer than those of others. For the fatty acid in loin, the contents of myristic acid, oleic acid and linolenic acid in Sika deer were higher than those of others, the contents of myristic acid, palmitoleic acid, oleic acid and arachidonic acid in Red deer were higher than those of others and in Elk, the contents of palmitic acid and stearic acid were higher than those of others. For the contents of unsaturated fatty acid and polyunsaturated fatty acids, the level was higher in Red deer than those of others. For the fatty acid in leg, the contents of oleic acid, linolenic acid, linolenic acid and arachidonine acid in Sika deer were higher than those of others, the contents of myristic acid, palmitic acid, palmitoleic acid and oleic acid in Red deer were higher than those of others and in Elk, the contents of stearic acid and linolenic acid were higher than those of others. For the contents of saturated fatty acid, the level was lowest in Sika deer. For the contents of unsaturated fatty acid and polyunsaturated fatty acids were respectively highest in Sika deer. Comparing the contents inorganic materials between loin and leg, the concentrations in leg were generally higher than that of loin.n.

• Journal of the Korean Society of Food Science and Nutrition
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• v.31 no.5
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• pp.885-892
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• 2002

The purpose of this research was to prove the excellency of unpolished (brown) rice with respect to nutritional composition and to compare chemical composition among six varieties of rice. Some nutritional composition in the Polished (well -milled) rice and unpolished (brown) rice of the six typical Korean varieties (Chucheong, Hwasung, Odae, Ilmi, Dongjin, Ilpum) were determined. Most of nutrients in the brown rice were much greater than those in the milled rice and there was significant difference among rice varieties in some chemical compositions. The major minerals of milled rices were Ca, 6～15 mg%;P, 91～125mg%;Fe, 0.3～1.2mg%; Na, 10～14mg%;K, 106～205 mg%; Zn, 1.0～1.8mg%; and Mg, 32～58 mg%. The mineral contents of brown rices wre Ca, 4～11mg%;P, 92～286mg%;Fe, 1.3～1.9mg%;Na, 12～15mg%;K, 243～320mg%;Zn, 1.5～2.3mg%;Mg, 112～140mg%. Major fatty acids in six rice varieties were linoleic acid, oleic acid and palmitic acid, which comprised of about 93% of total fatty acid. Amino acid analysis showed that aspartic acid, glutamic acid, arginine and leucine were major amino acids, whereas histidine, threonine and tryptophan were minor ones of rice. Most ammo acid contents was higher in Ilmi than the other varieties. The contents of vitamin in brown rices were considerably higher than those in well-milled rice. There was not significant difference in total dietary fiber among rice varieties : 1.1～1.2% for milled rice,3.2～3.5% for brown rice.

• KOREAN JOURNAL OF CROP SCIENCE
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• v.65 no.3
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• pp.222-230
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• 2020

Maize grain quality can be improved by raising lysine content, which is an essential amino acid present in insufficient quantities in normal maize. Maize varieties with such modifications are known as quality protein maize (QPM). To date, no Korean maize cultivars contain high amounts of lysine. To introduce quality protein maize to Korean cultivars, we crossed QPM CIMMYT maize lines (CML) with KS140, an elite inbred line used as a parent of several cultivars such as 'Gangdaok' and 'Pyeonganok'. We analyzed the phenotypic characteristics of F₁ plants as well as the protein contents, amino acids, and fatty acids profiles of the self-pollinated seeds of the F₁ hybrids, and evaluated the feasibility of CML as a source of QPM. Days to anthesis of the CML ranged from 78 to 90 days after planting (DAP), whereas a range of 81~87 was recorded for F₁ hybrids. The average days to anthesis was 85 for KS140, CML, and the F₁ hybrids. The protein content of the CML was measured to be between 9.1 and 12.1%, with the highest and lowest values being recorded in CML153 and 191, respectively. The F₁ hybrids had protein contents of 9.1~11.1%, and the highest content was observed in KS141/CML188. The fatty acids profiles were very similar across all analyzed maize samples, and linoleic acid (C18:2) composed the greatest proportion. Glutamic acid made up the largest proportion of amino acids in all maize samples. Lysine composition was highest in CML155 (6.92% of all amino acids), with an average composition of 4.83% across the CML. In contrast, KS140 showed a lysine content of 2.51%. In F₁ hybrids, the average lysine composition was 3.46%, and KS140/CML164 (4.18%) and KS140/CML163 (4.99%) contained more lysine than either parent. Taken together, these results indicate that CML could become promising QPM sources to improve grain quality in Korean maize cultivars.

• Korean Journal of Food Science and Technology
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• v.15 no.1
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• pp.12-18
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• 1983

Changes in the quality and amino acid composition of soymilk prepared from soaked and germinated soybeans were investigated. Soybeans were soaked in water for 3 hrs and germinated at $18{\pm}1^{\circ}C$ for 5 days followed by water extraction at room temperature, and then the soymilk was boiled for 30 min. The initial yields of total solid and protein after soaking were 80.7% and 88.6%, respectively and decreased slowly during germination. A slow decrease in lipids and a rapid reduction in total sugar content were found during germination. The change in protein fraction content of soymilk showed an initial increase followed by a gradual decrease. The intrinsic viscosity increased rapidly after 3 days of germination to maximum value at 4th day, then decreased. The amino acid composition of protein fraction of soymilk showed little change while that of nonprotein fraction changed significantly. After 4 days of germination, aspartic acid and alanine increased more than twice, and methionine and tyrosine decreased to half of their initial composition in soymilk prepared from soaked soybean.

• Journal of Sericultural and Entomological Science
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• v.47 no.1
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• pp.12-17
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• 2005

To develop technique for the production of P. tenuipes stromata on a large scale, the infection of P. tenuipes and the growth of stroma were investigated by silkworm (Bombyx mori) variety. Also, studied about biological activites of fruiting body formed on silkworm. Infection rate of the 5th instar larvae of the silkworm with P. tenuipes was the highest in Yangwonjam, followed by Hachojam, Baegokjam and Chilbojam in that order. Also, as the inoculation times was increased, infection rate tended to be raised. The rate of fruiting body formation of the silkworm pupae infected with P. tenuipes was the highest in Baegokjam, followed by Yangwonjam and Chilbojam in the order. But, actually the fruiting body formation of the 5th instar larvae of the silkworm tested was good in Chilbojam, followed by Yangwonjam and Baegokjam in that order in 3 times spraying inoculation. The fruiting bodies of Yangwonjam and Chilbojam infected with P. tenuipes had high amount of Mannitol, but Baegokjam and Hachojam had high concentration of Glucose on a dry weight basis. The mean content of total amino acid in the fruiting bodies of P. tenuipes was 1.03 ${\mu}mole/g$. The distribution rate of amino acid components decreased in the order of Arginine (12.2%)>Glycine (10.5%)> Proline (9.6)>Tyrosine (8.9%)>Serine>Leucine>Threonine. The most abundant amino acid in the fruiting bodies of the Baegokjam, Chilbojam and Hachojam infected with P. tenuipes was arginine, while Yangwonjam was Glycine. The most abundant fatty acid in P.tenuipes was Oleic acid on a dry weight basis. The unsaturated fatty acids such as Oleic acid, Linoleic acid and Linolenic acid accounted for more than 78% of the total fatty acids.

• Korean Journal of Microbiology
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• v.49 no.3
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• pp.228-236
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• 2013

Campylobacter jejuni is one of important food-borne pathogens causing human gastroenteritis. We isolated 42 strains of C. jejuni from diarrhea patients and 4 food-poisoning outbreaks in 2010, Gyeonggi-do. In this study, 42 strains were tested for genetic characteristics, the serotype distribution and antimicrobial resistant rate. The presence of hipO (100%), cdtB (100%), and mutated gyrA (95.2%) genes was detected in C. jejuni by polymerase chain reaction (PCR). Detection of mutated gyrA gene correlated with ciprofloxacin resistance. Forty isolates had mutated gyrA gene and were actually resistant to ciprofloxacin. Furthermore, comparing the gyrA DNA sequence data, ciprofloxacin-resistant isolates had a mutation of the DNA sequence from ACA (threonine) to ATA (isoleucine). But 41 strains (97.6%) of patient isolates were susceptible to erythromycin and azithromycin. A total of 35.7% among 42 C. jejuni isolates were identified into 4 different serotypes. The serotype distribution of C. jejuni strains were shown to be HS2(B), HS3(C), HS4(D), HS19(O). To investigate the genotypes of C. jejuni isolated in Gyeonggi province, repetitive sequence polymerase chain reaction (rep-PCR) analysis and SmaI-digested pulsed-filed gel electrophoresis (PFGE) profile analysis were performed. From the PFGE analysis of 42 C. jejuni strains, 12 clusters of PFGE profile were obtained. On the other hand, 11 clusters of rep-PCR profile were obtained from 42 strains of C. jejuni.

• Journal of Nutrition and Health
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• v.18 no.4
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• pp.259-271
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• 1985

The purpose of this study was to develop supplementary foods for infants and young children in order to improve their nutritional status. Three formulas composed of rice, soybeans, fish, dry skim milk and sesame in varying proportions were studied. The three formulas, $RS_{1}S_{2}$, $RFS_{1}S_{2}$, and $RMS_{1}S_{2}$, were consisted of Rice(R), Soybean$(S_{1})$, Sesame$(S_{2})$ (60 : 35 : 5) , Rice, Fish(F), Soybean, Sesame (60 : 10 : 25 : 5) , and Rice, Dry Skin Milk (M), Soybean, Sesame (60 : 10 : 25 : 5), respectively. A proximate analysis and amino acid determination were made on the developed formulas. In the animal assay, growth rate, PER and FER were evaluated and biochemical analyses were also carried out. A storage test and the cost evaluation were also conducted. The summarized results are as follows : 1) The proximate composition of the three formulas were 7.3-7.4% of moisture, 15.9-21.5% of crude protein, 7.8-9.6% of crude fat and 2.5-2.8% ash. 2) The result of amino acid analysis showed that the 1st limiting amino acids of $RS_{1}S_{2}$ and $RFS_{1}S_{2}$ were lysine (amino acid score, 76.6) and threonine (amino acid score, 93.3), and that of $RMS_{1}S_{2}$ and the commercially prepared formula were sulfur containing amino acids (amino acid score, 82.0 and 54.4). When the contents of the amino acids of the three formulas were compared with mother's milk and cow's milk, the balance of the amino acid of each formula was superior to mother's milk but inferior to cow's milk. 3) In the animal assay, the growth rate of all groups increased gradually during the experimental period. 4) The C- PER, which was corrected on the basis of the casein PER of 2.5 was 2.99, 3.38 and 3.10 in the $RS_{1}S_{2}$, $RFS_{1}S_{2}$ and $RMS_{1}S_{2}$ respectively. The C- PER of $RFS_{1}S_{2}$ and $RMS_{1}S_{2}$ were Significantly (P<.05) higher than that of the casein. 5) The FER of the casein, $RS_{1}S_{2}$, $RFS_{1}S_{2}$, and $RMS_{1}S_{2}$ were 0.37, 0.39, 0.43 and 0.39, respectively. The FER of $RFS_{1}S_{2}$ and $RMS_{1}S_{2}$ were also significantly (P<.05) higher than that of the casein. 6) The concentrations of hematocrit, hemoglobin, total protein and albumin in the serum of the rats of all groups were not significantly different among groups. 7) The storage stability test showed that the total plate count (TPC), the coliforms count and the bacterial spore count in the ingredients were quiet low. However, after 30 and 60 days storage, the count in $RFS_{1}S_{2}$ increased and were higher at room temperature than refrigerated temperature. 8) In the cost evaluation, the cost of the developed formulas was \1,826-2,626 / kg. This was less than that of the commercially prepared formula (\3,300-4,073 / kg) and that of the imported formula (\4,250-8,720 / kg).