• Applied Biological Chemistry
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• v.1
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• pp.55-61
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• 1960

The amino acid compositions of the protein and the nonprotein fractions obtained from a marine brown alga, 'Undaria pinnatifida' were determined by use of Ion Exchange Column Chromatography. The protein nitrogen in the alga was about ten times of the nonprotein nitrogen. Nonprotein fraction obtained from the extraction with 80 percent ethanol contains considerable amount of tree citrulline. Alanine content in the alga was the highest (about 1 per cent in dry weight) and one third of which was found in free state. The amino acid composition of the alga was well balanced and the content of the essential amino acids were relatively higher, than soybean protein. In addition, several peptide like substances were fractionated from nonprotein fraction, in which one way identified as a naturally occurring new tripeptide composed of alanine, glutamic acid and aspartic acid, and the remaining unknown substances are under investigation for the further information.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.148-154
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• 2016

A urea cycle disorder is a condition caused by a defect of the enzymes in the urea cycle, and deficiency of ornithine transcarbamylase (OTC), which converts carbamoyl phosphate and ornithine into citrulline, is the most common type of the disorder. OTC deficiency induces the accumulation of precursors of urea, ammonia, and glutamine, leading to neurological symptoms including hypotonia, respiratory failure, seizure, lethargy, and coma and sometimes to death. Because OTC deficiency is inherited in an X-linked manner, typical symptoms such as vomiting, poor feeding, and lethargy appear mainly in male neonates. We recently had a case that presented with neonatal onset lethargy, vomiting, and apnea in a 4-day-old boy. He was diagnosed with OTC deficiency by biochemical phenotype, including hyperammonemia and an increased orotic acid level in the urine. Genetic analysis of the OTC gene showed a novel mutation c.780_781insCAGGCAGTGT (p.Ile261Glnfs*35). He was treated for hyperammonemia using continuous venovenous hemofiltration (CVVH) at 118 hours after birth. After 4 days of CVVH, his consciousness and blood ammonia concentration were normalized, and he was discharged at the age of 53 days. At around 12 months of age, bilateral femur fractures and osteomyelitis occurred in this patient. Two months after the fracture, he died of septic shock, insulin-resistant hyperglycemia, and multi-organ failure.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.186-190
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• 2014

Citrullinemia type 2 (citrin deficiency) is an autosomal recessive inborn error metabolism, caused by the SLC25A13 gene mutation. Citrin deficiency is associated with two clinical phenotype; neonatal-onset type II citrullinemia (CTLN2), also known as neonatal intraphepatic cholestasis caused by citrin deficiency (NICCD) and adult-onset CTLN2. Clinical manifestations of NICCD include poor growth, intrahepatic cholestasis, liver dysfunction and increased plasma citrulline, methionine, threonine, arginine. The molecular diagnosis could be confirmed by SLC25A13 gene mutation analysis. A 3-month-old male infant with persistent jaundice was referred for evaluation. Newborn screening was normal at birth. Mild elevation of serum ammonia and AST/ALT were observed. Plasma amino acid analysis showed significantly elevated citrulline, methionine, threonine. DNA sequence analysis of the SLC25A13 gene revealed two compound heterozygous mutations, c.[852_855del]($p.Met285Profs^*2$) and [1180+1G>A]. We suggest that NICCD should be considered as one of the cause of in infants with cholestatic jaundice, although the newborn screening was normal.

• Applied Biological Chemistry
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• v.47 no.4
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• pp.403-408
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• 2004

We studied active substances like crude cell wall components, crude protein, composing amino acids and free amino acids including orinithine cycle-related amino acids such as asparagine, ornithine and citrullin in fully ripe fruits of Korean native squash, Cucurbita moschata Poir. Crude protein content of 'Jeju 2' was the highest with $2,830\;{\mu}g/g$, while 'Sangju' was the lowest with $1,319\;{\mu}g/g$. Regarding the contents of crude cell wall components, fruit 'Kanghaw' was the highest with 2,961 mg% while 'Namhea' was the lowest with 1,582 mg%. Pectin contents of crude cell wall components were the highest in 'Kanghaw' (2,198 mg%) followed by 'Jeju 2' (2,178 mg%) and 'Jeju l' (1,461 mg%). The main contents of amino acids in squash were glutamic acid, aspartic acid, lysine, leucine and valine, which comprised to be more than 50% of total amino acid contents. Especially, in 'Jeju 2' aspartic acid and threonine were not detected. In fully ripe fruits, a total of 34 kinds of free amino acids were detected including 8 kinds of essential amino acids (histidine, isoleucine, leucine, lysine, phenylalanine, methionine, threonine and valine). More than 50% of the total free amino acids were aspartic acid and asparagine, and also all varieties were detected in ornithine, citrullin, and arginine, which are related to Ornithine cycle. There was a big difference in the contents of arginine in all varieties whereas the contents of ornithine and citrullin were very similar. 'Teaan' 29.34% was 7 times higher than 'Namhea' 4.30% in regards to arginine contents.

• Food Science and Preservation
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• v.13 no.6
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• pp.691-696
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• 2006

This study was carried out to analyze useful component in freeze-dried persimmon flower powder made from six cultivais. The cultivais were Dogunjosang, Kojongsi, Kabjubaeknok, Chalang, Weolhasi and SangjuDungsi. Powder of persimmon flower was prepared by milling after freeze drying at $-70^{\circ}C$. Crude lipid was the highest in Dogunjosang (57.26%). Major free sugars of the persimmon flowers were fructose ($95{\sim}310mg%$), glucose ($75{\sim}281mg%$) and sucrose ($7{\sim}28mg%$). Major organic acids were malic acid (225 mg% in Kabjubaeknok (Jun. 4th)) and tartaric acid (189 mg% in Kabjubaeknok (Jun. 4th)). Predominant free amino acids were hydroxy-L-proline(25.33 mg% in Weolhasi), L-citrulline (58.83 mg% in SangjuDungsi (May 280)) and L-threonine (11.88 mg% in SangjuDungsi (May 280)). Major phenolic compounds in the persimmon flowers were caffeic acid ($1,946{\mu}g/100 g$ in Kabjubaeknok (Jun. 4th)), p-hydioxybenzoic acid($418{\mu}g/100 g$ in SangjuDungsi (May 29th)) and protocatechuic acid($181{\mu}g/100 g$ in Kabjubaeknok(Jun. 1st)). The results suggest that persimmon flowers be potential materials as useful food ingredients.

• Food Science and Preservation
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• v.18 no.5
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• pp.746-754
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• 2011

This study analyzed the taste components of dorsal muscle and fin muscle collected from olive flounder cultured with extruded pellet (EP) compared with those of olive flounder cultured with raw fish moist pellet (MP) as a control. The olive flounder in this study were cultured for 10 months with either the formulated extrude pellets (FEP), commercial extruded pellets (CEP) or the MP feed, and the average weight was 1.15 kg. The major nucleotides and related compounds in the dorsal muscles of the fish fed with MP, CEP and FEP diets were AMP and IMP. The fin muscle of the olive flounder contained the largest amounts of IMP($5.91{\mu}mol/g$). The K value of the dorsal muscle in olive flounder fed with MP, CEP and FEP showed 9.63%, 9.83% and 5.84%, respectively. The free amino acids in the dorsal muscle of olive flounder showed significantly differences in the composition depending on the feed, but the distribution of free amino acids showed similar patterns. Among free amino acids, taurine showed the highest content in all the experimental groups, and the asparagine and citrulline contents were significantly higher in the groups fed with CEP and FEP than in the group fed with MP. The significant differences were observed in the contents of specific free amino acids in dorsal muscle and fin flesh depending on the feed for cultured olive flounder.

• Food Science and Preservation
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• v.10 no.3
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• pp.302-307
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• 2003

In the present study, we investigated the quality characteristic of old pumpkin extract treated with enzymes. As a results, all the groups treated with pectinase were better in quality characteristic than control group and the group treated with 0.15％(w/w) pectinase was specially great. All the groups treated with simultaneous pectinase and cellulase were higher in the extraction rate than the groups treated with pectinase or cellulase. The experimental groups were divided into non-treated control(I) and three treatment groups(II- IV) for optimum condition of enzyme treatment. The II and IV groups were treated with 0.15％(w/w) pectinase and 0.15％(w/w) cellulase, respectively, and the ill group was treated with both 0.15％(w/w) petinase and 0.05％(w/w) cellulase. Yield for old pumpkin extract of the III group (86.94％) was higher than that of other groups, but there were no significant difference among the groups in soluble solide content and pH of the extract. Reducing sugar and total sugar contents in the ill group were 2.81％ and 4.60％, respectively. Total carotene content in the II group (5.36 mg％) was higher than other groups. Old pumpkin extracts in all the groups showed nitrite-scavenging ability to pH 1.2, 3.0 and 4.0. Total free amino acid content in the III group (176.7 mg％) was higher than other groups. Citrulline contents in the II and III groups were detected 1.66 and 1.41 mg％, respectively but the contents in other groups were not detected.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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• pp.303-329
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• 2019

Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting "nutritional failure" which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.

• The Korean Journal of Food And Nutrition
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• v.36 no.1
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• pp.17-24
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• 2023

To apply UV-C as a non-heating sterilization method to increase the microbiological safety of fresh seedless watermelon products, reductions in E. coli and quality changes by treatment dose (0, 2, 4, 8, 14, 20 kJ/m²) were investigated. The pH, sugar content, and hardness of watermelon inoculated with E. coli were not significantly different according to the UV-C treatment dose, but the polyphenol content was significantly decreased compared to the controls (425.4 GAE ㎍/g F.W.). When treated with 2 and 4 kJ/m², the lycopene content was 31.6 and 30.9 ㎍/g F.W., respectively, which was increased compared to the controls (28.5 ㎍/g F.W.). The arginine and citrulline content was also significantly increased compared to the controls. The number of E. coli was significantly decreased compared to the controls following UV-C treatment. Considering the degree of E. coli reduction, lycopene content, arginine content, citrulline content, and UV-C irradiation time, subsequent experiments were conducted by selecting a UV-C treatment dose of 2 kJ/m². The results of confirming the degree of reduction in the number of E. coli colonies by a single treatment and combined treatment with UV-C 2 kJ/m² and 70% ethanol showed that the combined treatment was most effective as colonies were decreased by 2.3 log CFU/g compared to the controls. Therefore, it is judged that UV-C 2 kJ/m² radiation and combined treatment with 70% ethanol could be applied as a non-heating sterilization method for fresh watermelon slices.

• Journal of Photoscience
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• v.9 no.2
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• pp.205-208
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• 2002

Nitric oxide (NO) is a newly described transmitter involved with cell to cell communication that is generated in biologic tissues by specific types of nitric oxide synthase (NOS), which metabolize L-arginine and molecular oxygen to citrulline and nitric oxide. In the skin. NO has been reported to play an important role in such diseases as psoriasis, atopic dermatitis, and contact dermatitis, as well as act as an important modulator in UVB-induced erythema. Ultraviolet B irradiation to the skin evokes an increase in NO production in the epidermis through two pathways; induction of inducible NOS, mediated by inflammatory cytokines, and elevation of constitutive neuronal NOS activity. In a cell culture system, it has been demonstrated that NO functions as a melanogen after being produced in keratinocytes in response to UVB-irradiation. NO-stimulated melanogenesis in melanocytes is mediated by the cGMP/PKG pathway. In this study, up-regulation of tyrosinase gene expression by NO-stimulation and the involvement of NO in UVB-induced pigmentation were examined. In NO-induced melanogenesis, protein synthesis and tyrosinase activity increased along with an up-regulation of tyrosinase gene expression. In an animal model, UVB-induced pigmentation in skin was suppressed by sequential daily treatments with a specific inhibitor of NOS. Thus, NO plays an important role in UVB-induced pigmentation, where its function as a melanogen is considered to be one of the mechanisms. Together with its role in the development of erythema, NO contributes to the total protective response of skin against UVB-irradiation.