• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.2
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• pp.69-76
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• 2017

Citrin deficiency is an autosomal recessive disorder caused by mutations in the SLC25A13 gene on chromosome 7q21.3, and a type of urea cycle disorder that causes hyperammonemia. Although neonatal intrahepatic cholestasis and adult-onset type II citrullinemia, a type of citrin deficiency, have been described well in many articles for several decades, failure to thrive and dyslipidemia caused by citrin deficiency (FTTDCD), the other type of citrin deficiency, has been only identified recently. There was previously no case report about FTTDCD in Korea. Patients with FTTDCD could present with loss of appetite, fatigue, failure to thrive, hypoglycemia, hypercitrullinemia, dyslipidemia, and an increased lactate/pyruvate ratio. Routine evaluation may not reveal the cause of hypoglycemia caused by citrin deficiency. We recently had a case that presented with recurrent hypoglycemia in a 30-month-old boy. Chemistry profiling, urine organic acid analysis, plasma acylcarnitine analysis, and hormone studies indicated values within the normal range or non-specific findings. Mutation analysis to identify the cause of hypoglycemia identified the subject as a compound heterozygote carrying each of the c.852_855del ($p.Met285Profs^*2$), and c.1177+1G>A mutant alleles. We report here on this unusual case of citrin deficiency presenting with FTTDCD for the first time in Korea.

• Journal of Applied Biological Chemistry
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• v.59 no.3
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• pp.265-271
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• 2016

Recently, liver damage contributes to big percentage of the morbidity and mortality rates worldwide. Excessive intake of alcohol is one of the major causes of liver injury. When liver injury is repeated and becomes chronic, it leads to development of fibrosis and cirrhosis. In the liver, TGF-${\beta}$ is a profibrogenic cytokine, which participates in various critical events cause liver fibrosis. Seahorse (Hippocampus abdominalis) is a common traditional Chinese medicine and has been widely used for centuries. Seahorse has been known to have a variety of bioactivities, such as anti-oxidant, anti-fatigue, and anti-tumor. Peptide is one of the main compounds of seahorse. In this study, we isolated enzymatic hydrolysate from seahorse H. abdominalis by alcalase hydrolysis and investigated the effect of the hydrolysate on liver injury. In the present in vitro studies, the hydrolysate increases cell viability of Chang cells and protects Huh7 cells from ethanol toxicity. In addition, the hydrolysate inhibits TGF-${\beta}$-induced responses. In vivo studies show that the pretreatment of hydrolysate reduces alcohol-induced increases of serum Glutamic oxaloacetic acid transaminase and Glutamic pyruvate transaminase activities and increases liver weight and body weight. These results suggest that seahorse may have a hepatoprotective effect.

• Journal of Food Hygiene and Safety
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• v.24 no.3
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• pp.285-290
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• 2009

This study aimed to investigate the protective effect of Solanum nigum Linne total extract (SNT), Solanum nigum Linne leaf extract (SNL), Solanum nigum Linne root extract (SNR) on liver injury induced by Lipopolysaccharide(LPS) in Sprague-Dawley rats. SNT, SNL, SNR of 100 mg/kg concentration was intraperitoneally administered into rats at dose of 1.5 ml/kg for 20 days. on the day 1.5 ml/kg of LPS was injected. Four hours later, they were anesthetization with ether and dissected. Glutamate oxaloacetate transaminase (GOT), glutamate pyruvate transaminase (GPT) were measured in serum and superoxide dismutase (SOD), catalase and glutathione peroxidase (GPX) were measured in liver homogenate. SNT, SNL, SNR extract inhibited GOT and GPT activities in LPS-induced rats, whereas increased SOD, Catalase and GPX activity in liver tissue of LPS-induced rats. These suggested that SNT, SNL, SNR could be used for functional beverage.

• Korean Journal of Animal Reproduction
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• v.14 no.1
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• pp.1-8
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• 1990

These experiments were carried out to investigate the effects of cumulus cells for in vitro fertilization and development of bovine follicular oocytes matured in vitro. The bovine ovaries were obtained at a slaughter house and the follicular oocytes surrounded by cumulus cells were collected by puncturing follicles with 2~6 mm of diameter. Bovine oocytes were matured in vitro for 24~26 hours in a CO2 incubator with 5% CO2 in air at 39$^{\circ}C$. The medium used for maturation was TCM-199 supplemented with hormones, pyruvate, FCS and antibiotics. Epididymal spermatozoa were capacitated by in vitro culture for 2~3 hours in BO solution containing BSA(5mg/ml) and caffeine(2.5mM). Insemination was made by introducing about 10~15 matured oocytes into the suspension of capacitated spermatozoa. Six hour after insemination the eggs were transferred to TCM-199 supplemented with FCS(10%) and HEPES(25mM), cultured for 7~8 days with 10~15 eggs/well in 4-well multidishes(Nunc Co.) forming cumulus cell monolayer. The results obtained in these experiments were summarized as follows ; 1. The majority of the follicular oocytes with compacted cumulus cells existed in GV stage while those with dispersed or denuded cumulus cells existed GVBD and M II stage. 2. After 24~26 hours maturation, the maturation rates of the follicular oocytes cultured in TCM-199 containing hormones were slightly higher than those of oocytes cultured in medium without hormones, and the frequency of cumulus compacted or denuded oocytes reaching M II stage cultured in medium containing hormones was 75.7% or 51.7%, respectively(P<0.05). 3. After 20 hours in vitro insemination, percentages of ova fertilized were 61.4% or 51.4%, respectively, for cumulus oophorus intacted or removed, and increased frequency of ova with both male and female pronuclei was found when cumuli were present(P<0.05). 4. The rates of embryos developed to 2-, 4-, 8-, 16-cell and morula or blastocyst stage after cocultured with cumulus cells were 65.0%, 45.3%, 34.7%, 28.0% and 22.7%, respectively. The results for momla or blastocyst stage were significantly higher than those of the embryos cultured in the basic medium(P<0.05).

• Journal of Life Science
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• v.24 no.2
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• pp.118-127
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• 2014

The kinetic properties and isozyme expression of lactate dehydrogenase (EC 1.1.1.27; LDH) in tissues from Rana catesbeiana I and II collected from February (I) and August (II) were studied. LDH activities, A4 isozyme, and LDH/citrate synthase (EC 4.1.3.7; CS) were high in skeletal muscle from R. catesbeiana I, and LDH $B_4$ isozyme increased in several tissues of R. catesbeiana II. In particular, LDH activities were high in heart and brain tissues from R. catesbeiana II. LDH eye-specific C isozyme, detected by native polyacrylamide gel electrophoresis after immunoprecipitation, was expressed in eye tissue and was more similar to the $B_4$ than $A_4$ isozyme. LDH $A_4$ isozyme was purified by oxamate-linked affinity chromatography, and the molecular weight of subunit A was 32.0 kDa. In R. catesbeiana II, levels of $Km^{PYU}$, $Vmax^{LAC}$, and tolerance to lactate of LDH were high in all tissues, and $Vmax^{PYU}$ of LDH in heart and brain tissue was highly detected. Purified $A_4$ isozyme and LDH in eye tissue were highly tolerate compared to others. The $Km^{LAC}$ value was highly measured compared to $Km^{PYU}$. The degree of inhibition by 10 mM of pyruvate on LDH activities in tissues from R. catesbeiana I and II was more pronounced as the ratio of subunit B increased. As a result, characteristic expression of LDH eye-specific C was found in R. catesbeiana. Anaerobic metabolism seemed to predominate as the LDH of skeletal muscle from I showed higher activity. It also appeared that R. catesbeiana II adapted well to incremental increases in LDH B, becoming tolerant to the lactate of LDH in tissues.

• Journal of Life Science
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• v.19 no.2
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• pp.256-263
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• 2009

The lactate dehydrogenase (EC 1.1.1.27, LDH) $A_4$ isozyme in skeletal muscle of mandrin fish (Siniperca scherzeri) was successfully purified by affinity chromatography and ultrafiltration. The molecular weight of the purified LDH $A_4$ isozyme was 140.4 kDa and its isoelectric point (pI) was 7.0. Optimal pH for enzymatic reaction was 7.5. ${K_m}^{PYR}$ and $V_{max}$ value of the purified LDH $A_4$ isozyme were $4.86{\times}10^{-5}$ M and 13.31 mM/min using pyruvate as a substrate, respectively. These kinetic properties of the purified LDH $A_4$ isozyme supported the fact that the mandrin fish was a warm-adapted species. The antibody against the purified LDH $A_4$ isozyme may be used in the metabolic physiological studies of ectothermic vertebrates and in the diagnosis of several human diseases.

• Journal of Embryo Transfer
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• v.23 no.4
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• pp.263-267
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• 2008

The purpose of this study was to determine toxic effect of sucrose and trehalose prior to cryopreservation on nuclear maturation and embryonic development in immature bovine oocytes. All cryoprotectant was prepared in tissue culture medium 199-HEPES (TCM 199-HEPES) with 10% fetal bovine serum (FBS). Immature oocytes were exposed to 1.2M ethylene glycol (EG) and 0.1M sucrose or 1.2M EG and 0.1M trehalose for 3 min and then were exposed to 3.2 M EG and 0.25 M sucrose or 3.2 M EG and 0.25 M trehalose for 1 min. Oocytes treated with cryoprotectants were exposed to 0.25 M sucrose or 0.25 M trehalose for 5 min and then 0.1 M sucrose or 0.1 M trehalose for 5 min. Depending on type of sugar added to cryopreservation solution, oocytes were allocated to sucrose group and trehalose group, respectively. Oocytes exposed to TCM 199-HEPES with 10% FBS were considered as control. Oocytes were cultured in TCM 199 supplemented with 10% FBS, 5 ng/ml epidermal growth factor, 0.01 IU/ml luteinizing hormone, and $1\;{\mu}g/ml$ estradiol for 24 h in $39^{\circ}C$, 5% $CO_2$. Nuclear maturation was assessed by staining oocytes with 1% aceto-orcein. Oocytes were fertilized in vitro and were cultured in TCM 199 supplemented with 10% FBS, 5 mM sodium pyruvate, and antibiotics in $39^{\circ}C$, 5% $CO_2$. The rates of cleavage and blastocyst, and cell number in blastocyst were assessed. Metaphase II rates were not different among experimental groups regardless of type of sugar. The cleavage rate of trehalose group (73.3%) was significantly higher (p<0.05) than those of sucrose group (62.8%) and control group (60.8%). The blastocyst rate was significantly higher in trehalose group (p<0.05). Mean cell number in blastocyst were not different among experimental groups, although cell number of blastocyst in trehalose group was significantly higher on day 7 (p<0.05). In conclusion, sucrose and trehalose were not toxic to immature bovine oocytes prior to cryopreservation. In particular, trehalose was more effective on embryonic development.

• Journal of Pharmacopuncture
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• v.5 no.2
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• pp.76-91
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• 2002

The lipid lowering and antioxidant effects of Gansoo(BL18), Pungji(GB20) and Eumnungcheun(SP9) acupuncture in rats fed high fat diet were analyzed in biochemical and molecular biological aspects. The results obtained from this study are as follows : 1. In the body weight reduction, all acupuncture groups showed a high reduction compared to those of control group and in acupuncture groups, Gansoo(BL18) and Pungji(GB20) acupuncture groups showed a high reduction. 2. The concentration of plasma triglyceride, total cholesterol and LDL-cholesterol with acupuncture groups showed a little decrease and in acupuncture groups, Gansoo(BL18) and Pungji(GB20) groups showed a low values compared to those of other acupuncture groups. However, the tendency of HDL-cholesterol concentration showed no significant different. 3. The concentration of plasma ${\beta}-lipoprotein$ and free fatty acids showed a lowest values in the Gansoo(BL18) and Pungji(GB20) acupuncture groups and the glucose concentration showed to decrease in all treated acupuncture groups. 4. The concentration of liver total cholesterol and triglyceride in Gansoo(BL18) and Pungji(GB20) acupuncture groups showed a lower values than those of control group. 5. In all the acupuncture groups, the plasma glutamic oxaloacetic transaminase(GOT) activity showed a little decrease. In the glutamic pyruvate activity(GPT), Gansoo(BL18) and Pungji(GB20) acupuncture groups showed a lower values than those of control groups. However the values of eumneungcheun acupuncture only group showed no significant difference to those of control group. 6. The plasma and liver Thiobabituric acid reactive substance (TBARS) concentration in Gansoo(BL18) and Pungji(GB20) acupuncture groups were a lower than those of control group. However the values of eumneungcheun acupuncture group showed no significant difference to control group. 7. The superoxide dismutase (SOD) activity in Gansoo(BL18) acupuncture group and Glutathione peroxidase (GSH-Px) activity in Gansoo(BL18) and Pungji(GB20) acupuncture groups showed a high values. The catalase (CAT) activity in all the acupuncture groups showed a higher values than those of control group. 8. In acupuncture groups, DNA expression of Apo-B and Apo-E showed a tendency to decrease, however DNA expression of leptin showed no significant difference in all treatment groups. DNA expression of $TNF-{\alpha}$ showed a increase in acupuncture groups. These results indicate that Gansoo(BL18) and Pungji(GB20) (especially Gansoo(BL18)) acupuncture affect the lipid metabolism and showed possibility of lowering adipose tissue mass and lipid peroxidation.

• Proceedings of the Korea Water Resources Association Conference
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• 2006.05a
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• pp.1415-1419
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• 2006

메탄을 탄소원 및 에너지원으로 이용하는 메탄산화균은 물질대사과정 중에 다량의 세포외 고분자물질인 Extracellular polymeric substances(EPS)를 생성하는데, EPS는 카르복실기와 같은 표면흡착 기능을 가지고 있어 생체흡착제로 사용이 가능하다. 따라서 본 연구에서는 메탄산화세균을 이용하여 중금속인 Cd의 흡착성능을 파악하여 활성슬러지의 흡착능과 비교하고, EPS 농도별, pH별 흡착량의 변화를 실험한 후 Freundlich 흡착모델식에 적용하여 흡착공정의 기본적인 설계인자를 도출하고자 하였다. 실험에 사용한 메탄산화세균은 매립지 복토층 상부 토양에서 분리하여 실험실에서 대량으로 배양하였으며, EPS 생성을 위해 메탄을 Head space의 20%를 주입하고 $30^{\circ}C$, 150rpm에서 질소원이 부족한 조건으로 48hr 동안 배양하였다. Cd의 흡착실험은 용액의 pH를 3에서 8까지 변화를 주면서 활성슬러지와 메탄산화세균의 시간별 흡착능을 측정하였다. 또한 중금속의 농도별 흡착능을 측정하여 흡착평형 상수를 파악하였으며, 중금속 흡착 전, 후 미생물의 SEM 촬영, FT-IR 분석, 전자현미분석(EPMA)을 통하여 무기성분 분석 및 표면관찰을 수행하였다. 실험결과 메탄산화세균에 의해 생성된 EPS 물질은 중금속에 대한 강한 결합능력이 있으며, Cd에 대한 최고 흡착능은 26mg Cd(Ⅱ)/g VSS의 값을 보였다. 이러한 미생물의 EPS의 흡착능은 pH와 칼슘이온의 영향을 많이 받았으며, 메탄산화세균의 FT-IR 분석결과 EPS에는 sulfate ester, pyruvate 등과 같은 작용기와 amino sugar, carboxyl 작용기들이 많이 존재하여 활성슬러지에 비해 중금속의 흡착능이 높은 것으로 사료되었다.X>${\mu}_{max,A}$는 최대암모니아 섭취률을 이용하여 구한 결과 $0.65d^{-1}$로 나타났다.EX>$60%{\sim}87%$가 수심 10m 이내에 분포하였고, 녹조강과 남조강이 우점하는 하절기에는 5m 이내에 주로 분포하였다. 취수탑 지점의 수심이 연중 $25{\sim}35m$를 유지하는 H호의 경우 간헐식 폭기장치를 가동하는 기간은 물론 그 외 기간에도 취수구의 심도를 표층 10m 이하로 유지 할 경우 전체 조류 유입량을 60% 이상 저감할 수 있을 것으로 조사되었다.심볼 및 색채 디자인 등의 작업이 수반되어야 하며, 이들을 고려한 인터넷용 GIS기본도를 신규 제작한다. 상습침수지구와 관련된 각종 GIS데이타와 각 기관이 보유하고 있는 공공정보 가운데 공간정보와 연계되어야 하는 자료를 인터넷 GIS를 이용하여 효율적으로 관리하기 위해서는 단계별 구축전략이 필요하다. 따라서 본 논문에서는 인터넷 GIS를 이용하여 상습침수구역관련 정보를 검색, 처리 및 분석할 수 있는 상습침수 구역 종합정보화 시스템을 구축토록 하였다.N, 항목에서 보 상류가 높게 나타났으나, 철거되지 않은 검전보나 안양대교보에 비해 그 차이가 크지 않은 것으로 나타났다.의 기상변화가 자발성 기흉 발생에 영향을 미친다고 추론할 수 있었다. 향후 본 연구에서 추론된 기상변화와 기흉 발생과의 인과관계를 확인하고 좀 더 구체화하기 위한 연구가 필요할 것이다.게 이루어질 수 있을 것으로 기대된다.는 초과수익률이 상승하지만, 이후로는 감소하므로, 반전거래전략을 활용하는 경우 주식투자기간은 24개월이하의 중단기가 적합함을 발견하였다. 이상의 행태적 측면과 투자성과측면의 실증결과를 통하여 한국주식시장에 있어서 시장수익률을 평균적으로 초과할 수 있는 거래전

• Journal of Nutrition and Health
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• v.29 no.1
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• pp.22-31
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• 1996

The purpose of this study was to investigate the effect of vitamin E and selenium on the antioxidative defense mechanism in the liver of streptozotocin(STZ)-induced diabetic rats. Sprague-Dawley male rats(120$\pm$10gm) were randomly assigned to one control and five STZ-diabetic groups. Diabetic groups were classified to STZ-0E (vitamin E free diet), STZ-40E(40mg vitamin E/kg of diet), STZ-400E(400mg vitamin E/kg of diet), STZ-S(0.5ppm Se/kg of diet) and STZ-400ES(400mg vitamin E and 0.5ppm Se/kg of diet) according to the level of vitamin E and selenium supplementation. Diabetes was experimentally induced by intravenous adminstration of 55mg/kg of STZ in citrate buffer(pH 4.3) after 4-weeks feedng of six experimental diets. Animals were sacrificed at the 4th day of diabetic states. Activities of the serum glutamic oxaloacetate transaminase(GOT) and the glutaminc pyruvate transaminase(GPT) in STZ-0E, STZ-40E and STZ-S rats were higher than those of control. Liver xanthine oxidase activities were similar to serum GOT and GPT. Liver superoxide dismutase(SOD) activities were higher in STZ-0E and STZ-40E groups by 33%, 22%, respectively than that of control. Glutathione S-transferase(GST) activities of liver were similar to GSH-Px activities. The contents of vitamin E in liver tissue were significantly lower STZ-0E, STZ-40E and STZ-S groups by 50%, 36%, 45% than that of control. Reduced glutathione(GSH) contents of liver were lower STZ-0E, STZ-40E, STZ-400E, STZ-S and STZ-400ES groups by 57%, 51%, 19%, 18%, 12% than that of control. Lipid peroxide values (LPO) in liver were higher 5.6, 2.3 and 2.3 times in STZ-0E, STZ-40E and STZ-S group than that of control. The present results indicate that STZ-induced diabetic rats are more sensitive to oxidative stress, leading to the acceleration of lipid peroxidation process, which can be more accelerated by feeding the low level of dietary vitamin E. In the coincident supplementation of high dietary vitamin E and selenium antioxidative enzymes activities and physiolosical antioxidants were increased more than those of the separate supplementation of vitamin E or selenium. Therefore, dietary vitamin E and selenium reduced peroxidative damage of tissue, promoting antioxidative defense mechanism against lipid peroxidation by diabetes.