• Title/Summary/Keyword: Liver enzyme

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Clinical Features, Response to Treatment, Prognosis, and Molecular Characterization in Korean Patients with Inherited Urea Cycle Defects

  • Yoo, Han-Wook;Kim, Gu-Hwan;Seo, Eul-Ju
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.2 no.1
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    • pp.77-79
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    • 2002
  • The urea cycle, consisting of a series of six enzymatic reactions, plays key roles to prevent the accumulation of toxic nitrogenous compound and synthesize arginine de novo. Five well characterized diseases have been described, resulting from an enzymatic defect in the biosynthesis of one of the normally expressed enzyme. This presentation will focus on two representative diseases; ornithine transcarbamylase(OTC) deficiency and citrullinemia(argininosuccinate synthetase deficiency). OTC deficiency is one of the most common inborn error of urea cycle, which is inherited in X-linked manner. We identified 17 different mutations in 20 unrelated Korean patients with OTC deficiency; L9X, R26P, R26X, T44I, R92X, G100R, R141Q, G195R, M205T, H214Y, D249G, R277W, F281S, 853 del C, R320X, V323M and 10 bp del at nt. 796-805. These mutations occur at well conserved nucleotide sequences across species or CpG hot spot. The L9X and R26X lead to the disruption of leader sequences, required for directing mitochondrial localization of the OTC precursor. Their phenotypes are severe, and neonatal onset. The G100R, R277W and V323M mutations were uniquely identified in patients with late onset OTC deficiency. The other genotypes are associated with neonatal onset. Out of 20 patients with OTC deficiency, only 6 patients are alive; two were liver transplanted, and normal in growth and development at 2, 4 years after transplantation respectively. Citrullinemia is an autosomal recessive disease, caused by the mutations in the argininosuccinate synthetase(ASS) gene. We identified in 3 major mutations in 11 unrelated Korean patients with citrullinemia; G324S, $IVS6^{-2}$ A to G, and 67 bp ins at nt 1125-1126. Among these, the 67 base pair insertion mutation is novel. The allele frequency of each mutation is; G324S(45%), IVS6-2 A to G(32%), and 67 base pair insertion(14%). All patients are diagnosed at neonatal or infantile age. Interestingly, two patients presented with stroke like episode. Out of 11 patients, 5 patients died. Among 6 patients alive, one patient was successfully liver transplanted.

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Effect of Soy Protein Hydrolyzate on Lipid Metabolism and Antioxidant Activity in the Rat (대두단백가수분해물이 흰쥐의 지질대사와 항산화에 미치는 영향)

  • Han, Yoon-Hee;Park, Sang-Kyu;Kim, Hye-Young
    • Journal of Nutrition and Health
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    • v.41 no.2
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    • pp.119-126
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    • 2008
  • This study was conducted to investigate the effect of soy protein hydrolyzate on lipid metabolism and antioxidant activity in the rat. Thirty-eight male rats of Sprague-Dawley strain were divided into five groups: casein, isolated soy protein (ISP), seoritae protein hydrolyzate (SH), soluble soy protein hydrolyzate (SS), and insoluble soy protein hydrolyzate (IS). The control diet (casein group) contained 20% casein protein and experimental diet contained 10% casein and 10% isolated soy-protein or soy-protein hydrolyzate. Fecal lipid content was increased and lipid apparent absorption rate was decreased significantly by the ISP group at the first week of experimental period. Blood triglyceride, total cholesterol, LDL-cholesterol and atherogenic index (AI) were decreased by soy protein hydrolyzate groups than casein group. Liver total lipid, triglyceride and cholesterol were not different among groups, but showed decreasing tendencies in soyprotein hydrolyzate groups. The lipid lowering effect was prominent in the IS group among soy protein hydrolyzate groups. Total antioxidant activity showed increasing tendency in the seoritae hydrolyzate group. Liver superoxide dismutase (SOD), glutathione peroxidase (GPx) and catalase activities also showed higher tendencies in the seoritae hydrolyzate group than other groups. In conclusion, insoluble soyprotein hydrolyzate was more effective in lowering body lipids and seoritae hydrolyzate had higher antioxidant capacity among soy protein hydrolyzates.

Effect of Meat Supplementation of Jeju Native Black Pigs Fed Tangerine Byproduct on Lipid Metabolism, Protein Level and Enzyme Activities in Rats (감귤 부산물을 급여한 제주 재래돼지고기의 섭취가 흰쥐의 지질대사, 단백질 농도 및 효소 활성에 미치는 영향)

  • Koh Jin-Bog;Yang Seung-Joo;Jung In-Chul;Ryon Jae-Suk;Moon Yoon-Hee
    • Food Science of Animal Resources
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    • v.26 no.2
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    • pp.175-182
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    • 2006
  • Diets consist of two different pork samples: pork of a Jeju native pig ( 260 days old, $101{\sim}103kg$ ) not fed tangerine byproduct during finishing period ($T_0$), and pork fed 8% and 15% tangerine byproduct during growing and finishing period ($T_1$), respectively. The effects of the diet on the physiological activities of rats were studied by feeding 17-week old rats with the two diets for 4 weeks. There was no significant difference between $T_0$ and $T_1$ in the rat's feed intake, feed efficiency ratio, and weight gain. Furthermore, there was no significant difference between $T_0$ and $T_1$ in the rat's weight of liver, kidney, spleen, epididymal fat pad, triglyceride and cholesterol of liver. Both $T_0$ and $T_1$ showed similar trends in terms of total lipid, phospholipid, triglyceride, total cholesterol, atherogenic index, protein, glucose, hemoglobin level, mineral level, and ${\gamma}$-GTP, ALT, AST and ALP activities. However, $T_1$ showed the trend of increasing amount of the serum's HDL and LDL cholesterol level, compared with $T_0$.

Reduction of Oxidative Stress by Chondroitin Sulfate in the Ovariectomy-Induced Aging Rat (난소절제로 유도한 노화쥐에서 chondroitin sulfate에 의한 산화 스트레스의 감소효과)

  • 이진영;하배진
    • Journal of Life Science
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    • v.14 no.2
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    • pp.280-285
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    • 2004
  • The ovarian hormone-deficiency induced ovariectomy rat is widely used as an aging model due to its practicality, convenience, and cost effectiveness. The surgically ovariectomized rat induces reactive oxygen species (ROS) generation like aging phenomena. Free oxygen radicals have been proposed as important causative agents of aging. The purpose of this study was to investigate the effect of chondroitin sulfate (CS) to prevent ovariectomy (OVX)-induced oxidative stress. The OVX rats were given intraperitoneally CS at doses of 100 mg/kg and 200 mg/kg daily for fifteen weeks. Malondialdehyde (MDA) levels were determined as well as the activities of superoxide dismutase (SOD), catalase (CAT), reduced-glutathione (GSH), oxidized-glutathione (GSSG), glutathione peroxidase (GPx) in the liver. The liver antioxidative enzyme activity was elevated while MDA concentration decreased in all CS treated animals. The results demonstrated that CS reduced oxidative stress in a dose dependent manner. These results suggest that CS might be a useful candidate for antioxidative reagent.

Effects of zinc bearing palygorskite supplementation on the growth performance, hepatic mineral content, and antioxidant status of broilers at early age

  • Yang, Weili;Chen, Yueping;Cheng, Yefei;Wen, Chao;Zhou, Yanmin
    • Asian-Australasian Journal of Animal Sciences
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    • v.30 no.7
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    • pp.1006-1012
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    • 2017
  • Objective: This study was conducted to investigate effects of zinc (Zn) bearing palygorskite (ZnPal) supplementation on growth performance, hepatic mineral content, and antioxidant status of broilers at early age. Methods: A total of 240 1-day-old Arbor Acres broiler chicks were allocated into 5 treatments with 6 replicates of 8 chicks each. Birds in 5 treatments were fed a basal diet supplemented with 0 (Control group; Analyzed Zn content: 81 mg/kg), 20, 40, 60, and 80 mg/kg Zn as ZnPal for 21 days, respectively. Blood, liver and intestinal mucosa were collected at 21 days of age. Results: Treatments did not affect growth performance of broilers during the 21-day study (p>0.05). The contents of hepatic Zn and magnesium (Mg) were linearly increased (p<0.001) by ZnPal supplementation. ZnPal inclusion linearly (p = 0.007) reduced malondialdehyde (MDA) concentration in serum. The activity of total superoxide dismutase (T-SOD) in liver increased linearly (p = 0.001) with concentration of ZnPal in diet. ZnPal inclusion linearly (p = 0.036) and quadratically (p = 0.005) increased T-SOD activity, and linearly (p = 0.012) increased copper/zinc superoxide dismutase (Cu/Zn SOD) activity in jejunal mucosa. The maximum responses of hepatic and jejunal antioxidant enzymes activities (T-SOD and Cu/Zn SOD) were found when supplementing the basal diet with 60 mg/kg Zn as ZnPal. Furthermore, ZnPal supplementation quadratically (p = 0.001) increased Cu/Zn SOD activity in ileal mucosa, and its maximum activity was observed in the diet supplemented with 20 mg/kg Zn as ZnPal. Conclusion: ZnPal supplementation did not alter growth performance of broilers. Dietary ZnPal inclusion could increase concentrations of hepatic trace minerals (Zn and Mg) and inhibit lipid peroxidation by reducing serum MDA accumulation, with the optimal dosage of Zn from ZnPal being 80 mg/kg diet (analyzed Zn content in the diet: 165 mg/kg), and 60 mg/kg Zn as ZnPal (analyzed Zn content in the diet: 148 mg/kg) was the optimum dosage for broilers to achieve maximum antioxidant enzyme activities.

Correlation between Deoxycytidineuria and CdR-aminohydrolase Activity following X-Irradiation (X線照射에 따르는 Deoxycytidineuria와 CdR-aminohydrolase의 活性變化와의 連關性)

  • Man Sik kang;Rhee, Juong-Gile;Cho, Joong-Myung
    • The Korean Journal of Zoology
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    • v.18 no.4
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    • pp.163-172
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    • 1975
  • This work was undertaken to elucidate some aspects of mechanisms underlying the increased deoxycytidineuria following irradiation in the mice, by observing Dische-positive substances liberated from thissues, the activity of CdR-aminohydrolase of tissues and the CdR excreted in the urine at various times after single whole-body exposure to 400 and 800 R of X-rays. The activity of CdR-aminohydrolase declined markedly at 1 hour in the small intestine and liver, followed by a gradual rise reaching a maximum at 3 days after irradiation. In the case of the spleen and blood, however, only a trace of activity was observed in the control and irradiated animals. The amount of Dische-positive substance liberated from the small intestine postirradiation was elevated from 3 to 12 hours, showing a maximum during 6 to 9 hours after irradiation. On the contrary, the activity of the enzyme in the liver, spleen and kidney was less than one twentieth that of the small intestine, suggesting a prediction that these organs are not attributable to the increased deoxycytidineuria. A maximum deoxycytidineuria was exhibited at 9-12 hours period, attributed a large amount of CdR to the small intestine, which might correlate with the change in the CdR-aminohydrolase activity. Radiation-induced CdR seems to be liberated from the small intestine into the blood when the CdR-aminohydrolase activity declines abruptly. Then, the CdR is rapidly subjected to a filtration in the kidney without undergoing a further degradation pathway in the blood.

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A seroepidemiological Study of Hepatitis B and C Virus (HBV and HCV) Infections in the Young Population in parts of Busan, Korea (일개 도시 일부 청년층(16-24세)의 B형, C형 간염에 관한 혈청역학적 연구)

  • Ju, Young-Hee;Oh, Jin-Kyoung;Kim, Dong-Il;Lee, Duk-Hee;Kim, Byeong-Kweon;Kim, Jung-Il;Jung, Kap-Yeol;Shin, Hai-Rim
    • Journal of Preventive Medicine and Public Health
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    • v.37 no.3
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    • pp.253-259
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    • 2004
  • Objectives : To investigate the prevalence of hepatitis B and C virus infections and determine the associated risk factors among young adults in Busan, Korea, which is known to have a high incidence of liver cancer. Methods : The study population consisted of volunteer participants in a health survey during 2002, which included 1,350 students (515 males and 835 females) aged between 16 and 24 years, from three different schools in Busan. The participating students were asked to fill in a self-administered questionnaire which included lifestyle habits and risk factors of hepatitis. Sera obtained from the participants were studied for HBsAg, anti-HBs, and Anti-HCV by enzyme immunoassay (EIA) method and for liver function tests. Results : Among the study subjects (N=1,350), the seropositivities of HBsAg 7.9%(95% CI=7.8-8.0), 7.6%(95% CI=7.6-7.7) in male and 8.1%(95% CI=8.0-8.2) in female. And the seropositivity of Anti-HBs was 69.7%(95% CI=69.0-70.4), 70.5%(95% CI=69.8-71.2) in male and 69.2%(95% CI=68.5-69.9) in female. The seropositivity of Anti-HCV was 0.4%, 0.2% in male and 0.5% in female. The seropositivity for HBsAg in the subjects not having a hepatitis B vaccination history was twice(95% CI=1.0-4.4) that of those that did. Also, the seropositivity for HBsAg in subjects having experienced sexual intercourse was 1.7 times (95% CI=0.9-3.0) that of the subjects who had not. Conclusions : The present study confirmed the high prevalence of HBsAg seropositivity and sexual transmission of HBV among adolescents and young adults may occur. Further studies to evaluate the relationship between HBV vaccination and sexual transmission are required for the young population in Korea.

Should an Aberrant Left Hepatic Artery Arising from the Left Gastric Artery Be Preserved during Laparoscopic Gastrectomy for Early Gastric Cancer Treatment?

  • Kim, Jieun;Kim, Su Mi;Seo, Jeong Eun;Ha, Man Ho;An, Ji Yeong;Choi, Min Gew;Lee, Jun Ho;Bae, Jae Moon;Kim, Sung;Jeong, Woo Kyoung;Sohn, Tae Sung
    • Journal of Gastric Cancer
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    • v.16 no.2
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    • pp.72-77
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    • 2016
  • Purpose: During laparoscopic gastrectomy, an aberrant left hepatic artery (ALHA) arising from the left gastric artery (LGA) is occasionally encountered. The aim of this study was to define when an ALHA should be preserved during laparoscopic gastrectomy. Materials and Methods: From August 2009 to December 2014, 1,340 patients with early gastric cancer underwent laparoscopic distal gastrectomy. One hundred fifty patients presented with an ALHA; of the ALHA was ligated in 116 patients and preserved in 34 patients. Patient characteristics, postoperative outcomes and perioperative liver function tests were reviewed retrospectively. Correlations between the diameter of the LGA measured on preoperative abdominal computed tomography and postoperative liver enzyme levels were analyzed. Results: Pearson's correlation analysis showed a positive correlation between the diameter of the LGA and serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels on postoperative day 1 in the ALHA-ligated group (P=0.039, P=0.026, respectively). Linear regression analysis estimated the diameter of the LGA to be 5.1 mm and 4.9 mm when AST and ALT levels were twice the normal limit on postoperative day 1. Conclusions: We suggest preserving the ALHA arising from a large LGA, having diameter greater than 5 mm, during laparoscopic gastrectomy to prevent immediate postoperative hepatic dysfunction.

Direct Conversion of L-Selenomethionine into Methylselenol by Human Cystathionine ${\gamma}$-Lyase (인간 Cystathionine ${\gamma}$-Lyase에 의한 Selenomethionine의 Methylselenol로의 직접분해)

  • Cho, Hyun-Nam;Jhee, Kwang-Hwan
    • Microbiology and Biotechnology Letters
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    • v.42 no.1
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    • pp.11-17
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    • 2014
  • Selenium is an essential trace element for mammals, but it is very toxic. Therefore, the control of selenium concentrations should be precisely and effectively monitored. Selenium is naturally obtained through foods and seleno-L-methionine (LSeMet) is a major form of selenium. It has been reported that L-SeMet is only converted into Se-adenosyl-L-SeMet. However, a recent study suggested that L-SeMet was directly metabolized into methylselenol ($CH_3SeH$) in mouse liver extract by the reaction of cystathionine ${\gamma}$-lyase (CGL). The canonical reaction of CGL was known to catalyze the cleavage of L-cystathionine to L-cysteine, ${\alpha}$-ketobutyrate and $NH_3$. In the present study, we found that L-SeMet could be directly converted to $CH_3SeH$ using purified homogenous human CGL instead of mouse liver cytosol. Authentic $CH_3SeH$ was prepared by reduction of dimethyldiselenide with sodium tetrahydroborate. The gaseous product of the enzymatic reaction with L-SeMet was analyzed by GC/MS spectrometry. The GC/MS data was identical to that of authentic dinitrophenyl selenoether. We also analyzed the kinetic parameters for the formation of $CH_3SeH$ from L-SeMet by human and mouse CGL. These results suggest that human CGL is a critical enzyme which is responsible for L-SeMet metabolism.

Effect of Maengjong-Juk Extract Coated Rice Supplementation on Antioxidative System in Rabbit Fed High Cholesterol Diet (맹종죽 추출물 코팅쌀 식이가 고콜레스테롤 섭취 토끼의 항산화 시스템에 미치는 영향)

  • 이민자;김은영;문갑순
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.33 no.6
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    • pp.973-980
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    • 2004
  • To evaluate the antioxidative effect of rice coated with maengjong-juk extract in vivo system, rice coated with maengjong-juk extract diets were fed to NZW rabbit for 16 weeks and lipid peroxidation, protein oxidation, activities of antioxidative enzymes and total glutathione content in tissues were measured. TBARS contents in liver and spleen were significantly decreased in maengjong-juk extract diet group compared to control group, while those in kidney and heart tissue were not significantly different. Maengjong-juk extract diet suppressed the protein oxidation significantly in liver, spleen, kidney and heart tissues. Hepatic total SOD, Cu$.$Zn-SOD and Mn-SOD activities of maengjong-juk extract diets were significantly higher than those of control diet. GSH-Px and catalase activities of maengjong-juk extract diet were higher than those of control, while GR activities show no significant difference between the two groups. Total hepatic glutathione content was significantly increased by maengjong-juk extract diet. According to this study, many antioxidative materials and phytochemicals in maengjong-juk extracts seems to protect tissues from oxidative stress by stimulating antioxidative systems in atherosclerotic rabbit fed high cholesterol diet.