• Title/Summary/Keyword: glucose-6-phosphatase

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Heterogenous Clinical Manifestations in Adult Patients with Late Diagnosis of Glycogen Storage Disease type Ia (성인기에 진단된 당원병 제 Ia형의 다양한 임상 양상)

  • Kim, Yoo-Mi;Cheon, Chong Kun;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.1
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    • pp.9-17
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    • 2015
  • Glycogen storage disease (GSD) type Ia is rare inborn metabolic disorder, caused by glucose-6-phosphatase deficiency. It characterized by hepatomegaly, hypoglycemia, lactic acidosis, hypertriglyceridemia, and hyperuricemia and it is usually manifested in the infantile period. In addition, it is also associated with growth failure, pubertal delay, anemia, platelet dysfunction, osteopenia, and pulmonary hypertension. Hepatocellular adenoma and renal dysfunction are frequent late complications. Delayed diagnosis and inappropriate therapy lead to many complications such as growth failure, osteoporosis, refractory gout, renal failure, hepatocellular carcinoma (HCC), and pulmonary hypertension. Here, two Korean sisters diagnosed with GSD Ia, aged 33 and 36 respectively, were described and compared to recent articles about four adults with late diagnosis of GSD Ia. One sister had typical manifestations of GSD Ia including short stature (height, 145 cm), multiple hepatic adenoma, chronic kidney disease stage IV, and severe osteoporosis, whereas the older sister had normal stature (162 cm), one tiny hepatic nodule, and normal renal function. Direct sequencing of G6PC in two sisters identified a homozygous splicing mutation, c.645G>T, which is a prevalent mutation in Korea. Interestingly, our cases and four adults from recent reports had asymptomatic mild hypoglycemia and various manifestations including renal failure, HCC, fatty liver, or uncontrolled hyperlipidemia. These adult cases represent not only heterogenous phenotype to genotype within family members with GSD Ia but also long-term complications such as gouty arthritis, renal failure, and osteoporosis in untreated adult GSD Ia patients. In addition, lactic academia and hypertriglyceridemia are good markers of GSD Ia to distinguish from metabolic disease.

Suppressive Effects of Vitamin E on the Induction of Placental Glutathione S-transferase(GST-P) Positive foci and Antioxidant Enzyme Activity in Rat Hepatocarcinogenesis (비타민 E가 쥐간 세포의 암화과정에서 태반형 Glutathione S-transferase(GST-P) 양성 병소와 항산화요소계에 미치는 영향)

  • 최혜미
    • Journal of Nutrition and Health
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    • v.30 no.7
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    • pp.803-812
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    • 1997
  • The influences of dietary supplements of vitamin E on hepatocellular chemical carcinogenesis have been studied, Placental glutathione S-transferase(GST-P) positive foci area, antioxidant enzymes(superoxide dismutase(SOD), catalase, glutathione reductase, glutathione peroxidase, glutathione S-transferase(GST)), glucose 6-phosphatase(G6Pase) activities, and lipid peroxidation of mecrosomes(thiobarbituric acid reactive substances(TBARS) contents) were investigated. For is purpose , we used the murine chemical hepatocardinogenic procedure induced by modified Ito model, which consists of 200mg/kg body weight diethylinitrosamine (DEN) injection, 0.01% 2-acethlaminoflurene(2-AAF) feeding for 6 weeks, and partial hepatectomy on week 3. Weanling Sprague-Dawley male rats were fed pulverized Purina rat chow with 15, 000IU/kg diet vitamin E from initiation or promotion stages. We found that vitamin E supplement decreased the area of GST-P positive foci. Catalase, glutathione peroxidase, glutathione reductase. GST activities, and TBARS contents were decreased. On the other hand G6Pase activities were increased by vitamin E supplement. It seemed that vitamin E supplements helped endogenous defense systems against carcinogenesis by decreasing TBARS contents, $H_2O$$_2$ and organic peroxides. So, vitamin E seemed to protect cell from free radical damage in carcinogenesis. Anticarcinogenic effects of vitamin E were more effective at intiation that at promotion stage. These results suggest that vitamin E has suppressive effects on hepatocellular chemical carcinogenesis, probably through antioxidant effects against TBARS contents $H_2O$$_2$ and orgainc peroxides.

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Evaluation of Antidiabetic and Antihyperlipidemic Activity of Luffa tuberosa (Roxb.) Fruits in Streptozotocin Induced Diabetic Rats

  • Yeligar, Veerendra C.;Murugesh, K.;Dash, Deepak Kumar;Nayak, Siva Shankar;Maiti, B. C.;Maity, Tapan K.
    • Natural Product Sciences
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    • v.13 no.1
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    • pp.17-22
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    • 2007
  • Fresh unripe whole fruits of Luffa tuberosa (Roxb.) or Momordica tuberosa (Roxb.) Cogn. (Cucurbitaceae) were evaluated for the antidiabetic and hyperlipidemic potential in streptozotocin (STZ) induced diabetic rats. Diabetes was induced by administration of intra-peritoneal injection of streptozotocin at a dose of 55 mg/kg body weight. After the induction of diabetes aqueous extract of L.tuberosa (AELT) was administered orally at doses of 250 and 500 mg/kg. body weight/day for a period of 14 days. The Fasting blood glucose (FBG) levels, serum insulin levels, changes in body weight, food and liquid intake were measured. In diabetic rats, the AELT exhibited significant reduction in blood glucose levels. Biochemical assay of plasma total cholesterol (TC), triglyceride (TG), high-density lipoprotein cholesterol (HDLc), low-density lipoprotein cholesterol (LDLc), glycogen content and glucose-6-phosphatase activity in liver were assessed. Antihyperlipidemia in diabetic rats after the extract supplementation was confirmed by significant reduction in the levels of above mentioned hyperlipidemic indicators. This study focus on the efficacy of L.tuberosa fruits for the management of experimental STZ induced diabetic rats and provides the scientific basis of ancient herbal therapy and use of these fruits as vegetable.

Anti-hyperglycemic effects and signaling mechanism of Perilla frutescens sprout extract

  • Kim, Da-Hye;Kim, Sang Jun;Yu, Kang-Yeol;Jeong, Seung-Il;Kim, Seon-Young
    • Nutrition Research and Practice
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    • v.12 no.1
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    • pp.20-28
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    • 2018
  • BACKGROUND/OBJECTIVES: Perilla frutescens (L.) Britton var. (PF) sprout is a plant of the labiate family. We have previously reported the protective effects of PF sprout extract on cytokine-induced ${\beta}-cell$ damage. However, the mechanism of action of the PF sprout extract in type 2 diabetes (T2DM) has not been investigated. The present study was designed to study the effects of PF sprout extract and signaling mechanisms in the T2DM mice model using C57BL/KsJ-db/db (db/db) mice. MATERIALS/METHODS: Male db/db mice were orally administered PF sprout extract (100, 300, and 1,000 mg/kg of body weight) or rosiglitazone (RGZ, positive drug, 1 mg/kg of body weight) for 4 weeks. Signaling mechanisms were analyzed using liver tissues and HepG2 cells. RESULTS: The PF sprout extract (300 and 1,000 mg/kg) significantly reduced the fasting blood glucose, serum insulin, triglyceride and total cholesterol levels in db/db mice. PF sprout extract also significantly improved glucose intolerance and insulin sensitivity, decreased hepatic gluconeogenic protein expression, and ameliorated histological alterations of the pancreas and liver. Levels of phosphorylated AMP-activated protein kinase (AMPK) protein expression also increased in the liver after treatment with the extract. In addition, an increase in the phosphorylation of AMPK and decrease in the phosphoenolpyruvate carboxykinase and glucose 6-phosphatase proteins in HepG2 cells were also observed. CONCLUSIONS: Our results sugges that PF sprout displays beneficial effects in the prevention and treatment of type 2 diabetes via modulation of the AMPK pathway and inhibition of gluconeogenesis in the liver.

Effect of Carbaryl on the Toxicity of Fenvalerate in Rats (Fenvalerate의 독성에 미치는 Carbaryl의 영향)

  • 이상기;홍사욱
    • Environmental Analysis Health and Toxicology
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    • v.6 no.3_4
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    • pp.105-121
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    • 1991
  • The object of this study is to investigate the toxicity of fenvalerate [(RS)-$\alpha$-cyano-3 -phonoxybenzyl-(RS)-2-(4-ch1orophenyl)-3-methylbutyrate] and the effect of carbaryl on the toxicity of fenvalerate. Rats were treated with fenvalerate (50 mg/kg, 100 mg/kg), carbaryl (50 mg/kg, 100 mg/kg) or mixtures of the two compounds (fenvalerate+carbaryl: 50 mg/kg+50 mg/kg, 50 mg/kg+100 mg/kg) by oral administration for 1~3 weeks. Control groups were treated with corn oil. The experimental results were summarized as follows. 1. LD$_{50}$ values of fenvalerate and carbaryl in male rats were 385 mg/kg and 625 mg/kg respectively. When 50 mg/kg and 100 mg/kg of carbaryl were administratrd, LD$_{50}$values of fenvalerate were 265 mg/kg and 225 mg/kg respectively. 2. Biochemical parameters such as ALT, LDH and glucose in serum were much more increased in the groups treated with mixture than the groups treated with either one of fenvalerate or carbaryl. 3. The groups treated with carbaryl and mixture for 3 weeks, the contents of cytochrome P-450 in the liver were significantly increased. In renal microsomal fractions, however, no significant changes of drug metabolizing enzyme activities were observed. 4. The activities of aniline hydroxylase in hepatic microsomal fractions were increased in the groups treated with fenvalerate and mixture and activity was much more increased in the groups treated with mixture. 5. The activities of ATPase in the groups treated with fenvalerate were decreased than that of groups treated with mixture. TBA values and the activity of glucose-6 -phosphatase in the liver were not significantly changed. 6. In mixture treated groups, the activities of cholinesterase in serum and in the liver were more decreased than those of carbaryl treated groups. The activities of carboxylesterase in serum in the liver were slightly increased in mixture treated groups, but in fenvalerate treated groups, the activities of carboxylesterase were much more increased than those of control groups. 7. As a result of this study, when carbaryl was as the synergist of fenvalerate, carbaryl inhibited the activities of esterases, so the toxicity of fenvalerate was increased.sed.

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The Effects of Saururus chinensis Baill Extract Administration on the Blood Glucose, Electrolyte and Lipid Metabolism in STZ-Induced Hyperglycemic Rats (Streptozotocin 유도 당뇨성 흰쥐의 혈당, 전해질 및 지질대사에 삼백초 추출액이 미치는 영향)

  • Kim, Han-Soo
    • Journal of Life Science
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    • v.16 no.6
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    • pp.911-918
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    • 2006
  • The purpose of this study was to observe the effects of the feeding physiological activity substance in Saururus chinensis Baill extract on the improvement of the blood glucose, electrolyte (Na, K, CI) concentration and lipid metabolism in the serum of streptozotocin (STZ, 55 mg/kg B.W., I.P. injection)-induced hyperglycemic rats fed the experimental diets for 5 weeks. Concentrations of blood glucose, total cholesterol, atherosclerotic index, LDL, LDL-cholesterol, free cholesterol, cholesteryl ester ratio, triglyceride (TG) and phospholipid (PL) in serum were significantly higher in the hyperglycemic group (group NSW), STZ (I.P.)+Saururus chinensis Baill leaf 3.5 g% extract group (group NSSL) and STZ (I.P.)+Saururus chinensis Baill root 3.5 g% extract group (group NSSR) than those in the control group (group Normal, basal diet+water). But the concentrations of blood glucose, total cholesterol, atherosclerotic index, LDL, LDL-cholesterol, free cholesterol, cholesteryl ester ratio, TG and PL in serum were remarkably lower in the groups NSSL and NSSR than those in the NSW, whereas the ratio of HDL-cholesterol concentration to total cholesterol and HDL-cholesterol concentration in the groups NSSL and NSSR were significantly higher than in the group NSW. Electrolyte and creatinine concentrations in serum were significantly lower in the groups NSSL and NSSR than those in the group NSW. The activities of aminotransferase (AST, ALT), creatine phosphokinase (CPK), lactate dehydrogenase (LDH) and alkaline phosphatase (ALP) in serum were remarkably lower in the groups NSSL and NSSR than in the hyperglycemic group NSW. However, no significance was found in the comperative effect of the groups NSSL and NSSR. From the above results, it was suggested that Saururus chinensis Baill was effective in the improvement of the blood glucose, electrolyte, glucide and lipid metabolism in serum of STZ-induced hyperglycemic rats.

Sedative Effect and Changes in Blood Components in Cattle Given with Detomidine HCI (소에서 Detomidine 투여에 의한 진정효과 및 혈액성분의 변화)

  • Park Myeung-Ro;Nam Tchi-Chou;Kweon Oh-Kyeong
    • Journal of Veterinary Clinics
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    • v.6 no.1
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    • pp.209-216
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    • 1989
  • In order to investigate sedative action of detomidine and its effect on physical signs, hematological and blood chemical components, 15 Holstein cattle were used. The dosage of detomidine was 25 ${\mu}$g/kg and 50 ${\mu}$g/kg. Blood was collected before injection, 30, 60 and 120 min. after injection. Induction time of sedation in a cattle given with 25 ${\mu}$g/kg and 50 ${\mu}$g/kg of detomidine was 10.6${\pm}$2.8. 7.6${\pm}$1.0min. respectively and maintenance time was 70.4${\pm}$8.3, 86.5${\pm}$9.9, respectively. After injection of detomidine, body temperature was slightly increased, heart rate and respiratory rate were slightly decreased. The levels of red blood cell, hemoglobin, packed cell volume and white blood cell were not changed by detomidine. Blood glucose level following detomidine was markedly increased but total protein, serum glutamic oxaloacetic transminase, alkaline phosphatase, lactic dehydrogenase, blood urea nitrogen and creatinine were not changed. This results indicated that detomidine was useful sedative in bovine practice.

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Effect of Allopurinol Pretreatment on the Hepatic Xanthine Oxidase Activity in $CCl_4$-Treated Rats (흰쥐에 사염화탄소 투여시간 Xanthine Oxidase활성에 미치는 Allopurinol의 영향)

  • 윤종국;이혜자;이상일
    • Biomedical Science Letters
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    • v.1 no.1
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    • pp.37-43
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    • 1995
  • To evaluate an effect of xanthine oxidase(XO) reaction system on the carbon tetrachloride($CCl_4$) metabolism, $CCl_4$ was given twice at 0.1ml/100g body wt. at intervals of 18 hour to the rats and those pretreated with allopurinol (50mg/kg. body wt.). The influence of XO on the metabolism of $CCl_4$ was focused on the degree of liver damage and the activities of a $CCl_4$ metabolizing marker enzyme, glucose-6-phosphatase. The increasing rate of liver weight per body weight and the levels of serum alanine aminotransferase to the control group were more decreased in allopurinol-pretreated rats than in those treated with $CCl_4$ alone. The liver XO activities were more increased in $CCl_4$-treated rats than the control group and the $CCl_4$-treated rats pretreated with allopurinol showed a decreased activities of XO compared to the $CCl_4$-treated rats. The type conversion (type D --> type O) rate was more decreased tendency in allopurinol pretreated rats than those treated $CCl_4$ alone. In dialyzed liver enzyme preparations, all of the xanthine oxidase activities: $CCl_4$-treated, allopurinol and $CCl_4$-treated rats pretreated with allopurinol showed the more increased Vmax value than the control group, but similar Km value. Moreover, $CCl_4$-treated rats pretreated with allopurinol showed the more increased Vmax value than the group treated with $CCl_4$ alone. In conclusion, it can not be negate the possibility of metabolism of $CCl_4$ by the xanthine oxidase enzyme system.

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Influences of Rice and Barley straw Application in the Rice Rhizosphere (수도근권(水稻根圈) 환경(環境)에 미치는 볏짚과 보릿집 시용(施用)의 영향(影響))

  • Lim, Sang-Soon;Kim, Kwang-Sik
    • Korean Journal of Soil Science and Fertilizer
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    • v.21 no.4
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    • pp.434-442
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    • 1988
  • This study has been made to investigate the influences of organic matter on the soil composition, nitrogen fixing organism, soil enzyme activity and nitrogen fixing activity in the paddy rice rhizosphere when rice and barley straw were applied. The results are summarized as follows: 1. The pH in the submerged soil was increased from ear formation stage to harvesting. 2. In the rhizosphere, $Fe^{{+}{+}}$ content was decreased according to the growing stage, while increased in the nonrhizosphere. 3. In the initial stage, rhizosphere was higher than nonrhizosphere but in the late stage nonrhizosphere was higher than rhizosphere on the $NH_4-N$ content. 4. In the submerged soil, rhizosphere was higher than nonrhizosphere, on the concentration of glucose and pentose. 5. Changes of the number of nitrogen fixing organism in whole soil was not high. 6. Generally, rhizosphere was higher than nonrhizosphere on the soil enzyme activity such as phosphatase, ${\beta}$-glucosidase, and protease. 7. Acetylene-reducing activity was the highest in the tillering stage, and rhizosphere, Samgang (high-yielding variety) were higher than nonrhizosphere. Dongjin (general variety) respectively. 8. In the submerged soil applied barley straw, acetylene-reducing activity was slightly higher than rice straw in the initial stage.

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Effects of Long-Term Weight Training Exercise on Aging Heart in Rat (장기간 체중부하 운동훈련이 흰쥐 노화 심근에 미치는 영향)

  • 조현국;이영실;정형재;이용덕;박원학
    • Biomedical Science Letters
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    • v.4 no.2
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    • pp.87-101
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    • 1998
  • There is considerable interests in the effect of regular, vigorous exercise, in particular weight training as a possible measure in improving myocardial function. The present investigation aimed to examine possible effect of a long-term weight training program on the heart in aging rats. Male rats aged 3-,10-, and 20-month-old were divided at random into a control (sedentary) and the exercise group. The training group was exercised for 5 days a week by 1 RM of ten times with weight training apparatus. This investigation examined the changes of the heart muscle relative to histological, ultrastructural, cytochemical, and stereological studies in rats. Quantity of lipofuscin pigments was clearly increased in the weight training group of 15-month-old rats, and mitochondrial degeneration, vacuolization, and interstitial proliferation were observed as well. In the weight training group of 25-month-old rats the frequency as separated intercalated discs, fraying myofibrils and hypercontraction band increased in number compared with the same 15-mon1h-old group. From the experimental result of glucose-6-phosphatase activity, the enzyme activities decreased in the weight training group of 15-month-old rats, and more decreased in the same 25-month-old group. In stereological study, both 15- and 25-month-old training groups, mitochondrial and myofibrilar volume densities significantly decreased, whereas interstitial volume density significantly increased. From the experimental results obtained in the present study, it is suggested that long-term weight training exercise do not cause any significantly qualitative and quantitative ultrastructural change of the heart muscle in the young. On the contrary, long-term weight training exercise stress may actually induce degenerative changes in the heart muscle in the old age.

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