• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.3
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• pp.147-154
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• 2015

Purpose: 3HB and AcAc are two ketone bodies that can be used as energy source in brain via succinyl-CoA:3-ketoacid CoA transferase (SCOT) and mitochondrial acetoacetyl-CoA thiolase (beta-ketothiolase, T2), called ketolysis. In case of malfunction of these enzymes, ketolysis cannot occur fluently causing various clinical manifestations. We want to know the numbers of patients and clinical manifestations of ketolytic defects in Korea. Material: For 67 patients of ketolytic defects out of 2794 patients that have done urine organic acid analysis, we analyzed clinical manifestations and age distribution. The study period was from January 2007 to September 2015. Method: To confirm persistency of ketonuria, repeated and loading organic acid analysis were done at least 1 week period interval. SPSS was used for statistical analysis. Result: Thirty patients in infantile period (2 M-2 Y), 31 patients in childhood period (2 Y-12 Y), 5 patients after adolescent period (>12 Y) and 1 in neonatal period were diagnosed during the study period. The most frequent chief complaint was seizure followed by seizure with developmental delay and developmental delay only. Conclusion: Ketolytic defects were not so rare in Korea. Major clinical manifestations are seizure and developmental delay or mental retardation.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.1-8
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• 2005

Glutaric acidemia (GA) type II is a very rare inherited disorder that have no accruate figure on its icidende. People with Glutaric acidemia type II have an enzyme that does not work properly. Two specific enzymes are associated with Glutaric acidemia type II:1. Electron transfer flavoprotein (ETF), 2. ETF-ubiquinone oxidoreductase (ETF-QO). Both of these enzymes have similar functions in the body, and children with Glutaric acidemia type II may lack one or the other of these enzymes. They play an important role in breaking down fats and proteins, and help the body to produce energy. GA II clinically manifested as (1) neonatal onset with congenital anomalies (2) neonatal onset without anomalies, and (3) mild and/or later onset. The first two groups are sometimes said to have multiple acyl CoA dehydrogenation deficiency-severe and the third to have multiple acyl CoA dehydrogenation deficiency-mild. The course and age at presentation of later-onset glutaric acidemia type II is extremely variable, therefore it is difficult to diagnosis. We experienced one case of late onset form glutaric acidemia type II with afebrile status epilepticus-like convulsion.

• Food Science and Preservation
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• v.14 no.3
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• pp.323-327
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• 2007

Moderate consumption of maesil(Prunus mune) was associated with pharmaceutical and physiological effects on human health. The object of this study was to determine the inhibitory effects of Prunus mune extracts(PME) on food spoilage microorganisms. PME was found to have an antibacterial effect on Colletotrichum fragariae. The hydrophilic fractions of PME showed more effective inhibition than did the hydrophobic fractions. In addition, the hydrophilic fractions of PME seemed to inhibit(12-40%) metabolic enzymes related to energy production, including glucose-6-phosphate dehydrogenase, succinate dehydrogenase, malate dehydrogenase, and hexokinase. Our data suggest that hydrophilic components of PME might control food spoilage microorganisms because of suppression of membrane enzymatic function.

• Nutrition Research and Practice
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• v.10 no.1
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• pp.115-124
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• 2016

BACKGROUND/OBJECTIVES: This is the first study to identify common genetic factors associated with the basal metabolic rate (BMR) and body mass index (BMI) in obese Korean women including overweight. This will be a basic study for future research of obese gene-BMR interaction. SUBJECTS/METHODS: The experimental design was 2 by 2 with variables of BMR and BMI. A genome-wide association study (GWAS) of single nucleotide polymorphisms (SNPs) was conducted in the overweight and obesity (BMI > $23kg/m^2$) compared to the normality, and in women with low BMR (< 1426.3 kcal/day) compared to high BMR. A total of 140 SNPs reached formal genome-wide statistical significance in this study (P < $1{\times}10^{-4}$). Surveys to estimate energy intake using 24-h recall method for three days and questionnaires for family history, a medical examination, and physical activities were conducted. RESULTS: We found that two NRG3 gene SNPs in the 10q23.1 chromosomal region were highly associated with BMR (rs10786764; $P=8.0{\times}10^{-7}$, rs1040675; $2.3{\times}10^{-6}$) and BMI (rs10786764; $P=2.5{\times}10^{-5}$, rs10786764; $6.57{\times}10^{-5}$). The other genes related to BMI (HSD52, TMA16, MARCH1, NRG1, NRXN3, and STK4) yielded P < $10{\times}10^{-4}$. Five new loci associated with BMR and BMI, including NRG3, OR8U8, BCL2L2-PABPN1, PABPN1, and SLC22A17 were identified in obese Korean women (P < $1{\times}10^{-4}$). In the questionnaire investigation, significant differences were found in the number of starvation periods per week, family history of stomach cancer, coffee intake, and trial of weight control in each group. CONCLUSION: We discovered several common BMR- and BMI-related genes using GWAS. Although most of these newly established loci were not previously associated with obesity, they may provide new insights into body weight regulation. Our findings of five common genes associated with BMR and BMI in Koreans will serve as a reference for replication and validation of future studies on the metabolic rate.

• Korean Journal of Microbiology
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• v.55 no.3
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• pp.206-212
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• 2019

Propionibacterium acnes (P. acnes) lives in the hair follicles and pores, and it uses cell debris, sebum and metabolic byproducts of surrounding skin tissues as energy and nutrients. Increased production of sebum due to sebaceous hyperplasia or blockage of the follicle can cause growth and proliferation of P. acnes. The rapid growth of P. acnes in follicles produces cell damage, metabolic byproducts and bacterial chips, which can cause inflammation. In this study, we examined the possibility of Senecio iscoensis Hieron. (S. iscoensis) extract to regulate P. acnes-induced inflammatory signaling pathways. We observed that S. iscoensis extract effectively inhibited P. acnes-induced pro-inflammatory cytokine expressions such as IL-$1{\beta}$, TNF-${\alpha}$, and iNOS in mouse macrophage cell line Raw 264.7. The inhibitory effect of S. iscoensis in pro-inflammatory cytokine levels was accompanied by the inhibition of the transcription factors NF-${\kappa}B$ and NF-AT. However, S. iscoensis did not alter the P. acnes-induced MAPK signaling pathways. This study first suggests the potential of using S. iscoensis extract as an alternative agent for the treatment of acne.

• BMB Reports
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• v.54 no.5
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• pp.266-271
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• 2021

Estrogen-related receptor γ (ERRγ), a member of the orphan nuclear receptor family, is a key mediator in cellular metabolic processes and energy homeostasis. Therefore, ERRγ has become an attractive target for treating diverse metabolic disorders. We recently reported that ERRγ acts as a negative regulator of osteoclastogenesis induced by receptor activator of nuclear factor-κB ligand (RANKL). In the present study, we explored the effects of an ERRγ-specific modulator, GSK5182, on ERRγ-regulated osteoclast differentiation and survival. Interestingly, GSK5182 increased ERRγ protein levels much as does GSK4716, which is an ERRγ agonist. GSK5182 inhibited osteoclast generation from bone-marrow-derived macrophages without affecting cytotoxicity. GSK5182 also attenuated RANKL-mediated expression of cFos and nuclear factor of activated T-cells cytoplasmic 1 (NFATc1), pivotal transcription factors for osteoclastogenesis. Arrested osteoclast differentiation was associated with reduced RANK expression, but not with the M-CSF receptor, c-Fms. GSK5182 strongly blocked the phosphorylation of IκBα, c-Jun N-terminal kinase, and extracellular signal-regulated kinase in response to RANKL. GSK5182 also suppressed NF-κB promoter activity in a dose-dependent manner. In addition to osteoclastogenesis, GSK5182 accelerated osteoclast apoptosis by caspase-3 activation. Together, these results suggest that GSK5182, a synthetic ERRγ modulator, may have potential in treating disorders related to bone resorption.

• Journal of Nutrition and Health
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• v.56 no.5
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• pp.469-482
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• 2023

Purpose: Obesity has emerged as a critical global public health concern as it is associated with and increases susceptibility to various diseases. This condition is characterized by the excessive enlargement of adipose tissue, primarily stemming from an inequity between energy intake and expenditure. The purpose of this study was to investigate the potential of sweet pumpkin powder in mitigating obesity and metabolic disorders in leptin-deficient obese (ob/ob) mice and to compare the effects of raw sweet pumpkin powder (HNSP01) and heat-treated sweet pumpkin powder (HNSP02). Methods: Leptin-deficient obese mice were fed a diet containing 10% HNSP01 and another containing 10% HNSP02 for 6 weeks. Results: The supplementation of ob/ob mice with HNSP01 and HNSP02 resulted in decreased body weight gain, reduced adipose tissue weight, and a smaller size of lipid droplets in the adipose tissue and liver. Furthermore, the ob/ob-HNSP01 and ob/ob-HNSP02 supplemented groups exhibited lower levels of triglycerides, total cholesterol, low-density lipoprotein cholesterol, fasting blood glucose, and insulin, as well as a reduced atherogenic index in comparison with the control group. Molecular analysis also demonstrated that the intake of HNSP01 and HNSP02 resulted in a diminished activation of factors associated with fatty acid synthesis, including acetyl-CoA carboxylase and fatty acid synthase, while concurrently enhancing factors associated with lipolysis, including adipose triglyceride lipase and hormone-sensitive lipase, in the adipose tissue. Conclusion: Taken together, these findings collectively demonstrate the potential of sweet pumpkin powder as a functional food ingredient with therapeutic properties against obesity and its associated metabolic disorders, such as insulin resistance and dyslipidemia.

• Journal of Nutrition and Health
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• v.53 no.3
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• pp.231-243
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• 2020

Purpose: To determine the metabolic influence of the traditional Korean diet (K-diet), which has been regarded as a healthy diet, we investigated the profile of urine organic acids that are intermediates of various types of metabolism including energy metabolism. Methods: Ten women aged 50-60 years were recruited and randomly divided into 2 diet groups, K-diet and control diet, the latter of which is a Westernized Korean diet that is commonly consumed by Koreans nowadays. Before and after the 2-week intervention, 46 urine organic acids were determined using LC/MS/MS, along with clinical parameters. Results: The average concentrations of succinate (4.14 ± 0.84 ㎍/mg creatinine vs. 1.49 ± 0.11, p = 0.0346) and hydroxymethylglutarate (3.67 ± 0.36 ㎍/mg creatinine vs. 2.97 ± 0.29, p = 0.0466), both of which are intermediates of energy metabolism, decreased in the K-diet group after the 2-week intervention, but these were not observed in the control diet group. In particular, the average concentration of succinate in the K-diet group was lower than that in the control group (3.33 ± 0.56 ㎍/mg creatinine vs. 1.49 ± 0.11, p = 0.0284) after 2 weeks. The concentrations of two tryptophan metabolites, 5-hydroxyindolacetate (3.72 ± 0.22 ㎍/mg creatinine vs. 3.14 ± 0.21, p = 0.0183) and indican (76.99 ± 8.35 ㎍/mg creatinine vs. 37.89 ± 10.06, p = 0.0205) also decreased only in the K-diet group. After the 2-week intervention, the concentration of kynurenate, another tryptophan metabolite, was lower in the K-diet group than that in the control diet group (3.96 ± 0.51 ㎍/mg creatinine vs. 2.90 ± 0.22, p = 0.0356). Interestingly, the urine level of kynurenate was positively correlated with BMI (r = 0.61424, p = 0.0003) and total cholesterol (r = 0.46979, p = 0.0088), which decreased only in the K-diet group (239.40 ± 15.14 mg/dL vs. 198.20 ± 13.25, p = 0.0163). Conclusion: The K-diet alters the urinary excretion of organic acids involved in energy metabolism and tryptophan metabolism, suggesting the influence of the K-diet on these types of metabolism. Urine organic acids changed by the K-diet may serve as biomarkers in future studies.

• Applied Biological Chemistry
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• v.19 no.3
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• pp.172-183
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• 1976

RNA, DNA and other phosphorus fractions were determined in the leaf and root of soybean plants different in phosphorus sensitivity grown in $NH_4-N,\;NO_3-N$ and urea medium. The phosphorus sensitive cultivars contained higher ASIP (acid soluble inorganic phosphorus) than the tolerant cultivars with all nitrogen sources. ASIP was highest in the urea treated plants and lowest in the nitrate treated plants. Total phosphorus content was mostly affected with increase in ASIP. When ASIP increased, acid solsuble organic phosphorus(ASOP), phospholipids (L-P), RNA-P, residual phosphorus (R-P) tended to increase, while DNA-P showed little change. The percent RNA-P or DNA-P of total phosphorus in the nitrate treated plant was twice that in the ammonium treated plant, which were also higher in tolerant cultivars regardless of nitrogen sources. The percent ASOP in total acid soluble phosphorus $(ASOP/ASP{\times}100)$ decreased as phosphorus sensitivity decreased. Indications are that phosphorus sensitivity depends on the relative sizes of phosphorus metabolic pools. Total dry matter yield was negatively correlated with total phosphorus (r=0.84 significant at 0.01P), ASIP (0.84 significant at 0.01P) and residual phosphorus (0.69 significant at 0.05P). ASOP showed positive correlation with L-P, RNA-P and DNA-P but negative with R-P. RNA-P was significanly correlated only with L-P (0.63 at P=0.01). There was significant interaction (0.01) among nitrogen sources, cultivars and phosphorus metabolic pools. Phosphorus sensitivity and ammonium toxicity appear to be same in view of energy metabolism, that is, the former inhibits the conversion of ATP to ADP (energy releasing) through phosphate potential while the latter inhibits ATP formation (energy storing).

• Food Science of Animal Resources
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• v.30 no.1
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• pp.124-132
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• 2010

This study evaluated the effects of gamma ray and electron beam (E-beam) to improve the safety of spices for meat processing. The spices (garlic powder, curry powder, turmeric powder, black pepper, white pepper, oregano, parsley, laurel leaf powder, basil, and rosemary) were irradiated by gamma ray and E-beam at 0, 2, 4, 6, 8, and 10 kGy. Total bacterial populations were then enumerated on total plate count agar, and bacteria isolated from the samples were identified by polymerase chain reaction (PCR). In addition, $D_{10}$ values for Bacillus cereus and Staphylococcus aureus inoculated in spices was determined, and the Ames test was conducted for genotoxicity analysis. The contaminated total bacterial populations in spices ranged from 1.5 to 3.8 Log CFU/g, and most of identified bacteria were Bacillus spp., and Staphylococcus spp. However, the bacterial populations decreased below the detection limit (2 Log CFU/g) after irradiation at 4 kGy except for parsley, which required 6 kGy in gamma ray and 8 kGy in E-beam to decrease total bacterial populations below detection limit. $D_{10}$ values were also higher (p<0.05) in E-beam treated samples than gamma-ray treated samples. No genotoxicity was observed in both conditions with and without metabolic activation. These results indicate that gamma ray (>4 kGy and <6 kGy) could be more useful to improve food safety of meat processing spices compared to E-beam.