• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.2
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• pp.138-143
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• 2015

Glycogen storage disease type IX (GSD IX) is caused by a defect in phosphorylase b kinase (PhK) that results from mutations in the PHKA2, PHKB, and PHKG2 genes. Patients usually manifest recurrent ketotic hypoglycemia with growth delay, but some may present simple hepatomegaly. Although GSD IX is one of the most common causes of GSDs, its biochemical and genetic diagnosis has been problematic due to its rarity, phenotypic overlap with other types of GSDs, and genetic heterogeneities. In our report, a 22-month-old boy with GSD IX is described. No other manifestations were evident except for hepatomegaly. His growth and development also have been proceeding normally. Diagnosed was made by histologic examination, an enzyme assay, and genetic testing with known c.3210_3212del (p.Arg1070del) mutation in PHKA2 gene.

• Korean Journal of Veterinary Research
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• v.32 no.3
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• pp.357-364
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• 1992

Artemisia Iwayomogi Compositae) has been used clinically for jaundice, hepatitis, liver cirrhosis etc. The purposes of present study were to examine pharmacological effects of Artemisia Iwayomogi water extract(AIWE) on biochemical parameters (activities of ALP and LAP, contents of glucose, total bilirubin, total protein and albumin in serum, A/G ratio, and levels of hepatic glycogen) against hepatic injury by carbon tetrachloride($CCl_4$) in rats. The results were as follows ; 1. Increased ALP activities by $CCl_4$ were very significantly(p<0.001) decreased in AIWE posttreatment groups at 72 hours and significantly(p<0.05) decreased in AIWE pretreatment groups at 72 hours. Increased LAP activities by $CCl_4$ were significantly (p<0.05) decreased in AIWE posttreatment groups at 72 hours. A little increased total bilirubin contents by $CCl_4$ were very significantly (p<0.001) decreased in AIWE posttreatment groups at 24, 48 and 72 hours. 2. Increased glucose contents by $CCl_4$ were decreased in AIWE posttreatment groups. Decreased hepatic glycogen levels by $CCl_4$, were significantly (p<0.05) increased in AIWE posttreatment groups at 48 and 72 hours. 3. Decreased total protein contents by $CCl_4$ were significantly (p<0.05) increased in AIWE posttreatment groups at 48, 72 hours. Decreased albumin contents by $CCl_4$ were increased in proportion to numbers of AIWE treatments in AIWE pre- and posttreatement groups. Decreased A/G ratios by $CCl_4$ were significantly (p<0.05) increased in AIWE posttreatment groups at 48 hours. In conclusion, AIWE did not affect normal liver function and had hepatoprotective effects rather than direct preventive effects to $CCl_4$-induced cholestasis, damages in metabolisms of glucose, protein and bilirubin.

• BMB Reports
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• v.29 no.1
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• pp.81-87
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• 1996

The relationship of S-thiolation and oxidation of glycogen phosphorylase b and peroxidation of phosphatidyl choline liposome by xanthine oxidase (XOD), 2,2'-azobis(2-amidinopropane) hydrochloride (AAPH), and 2,2'-azobis(dimethylvaleronitrile) (AMVN)-generated free radicals was investigated, Glycogen phosphorylase b was S-thiolated in the presence of glutathione and oxidized in the absence of it by XOD, AAPH and AMVN. In XOD-initiated reaction, the rates of S-thiolation and oxidation of phosphorylase were very similar and addition of liposome to the reaction mixture showed little inhibition of the modifications. In AAPH-initiated reaction, the rate of oxidation was higher than that of S-thiolation and addition of liposome increased oxidation of the protein but had no effect on S-thiolation. In AMVN-initiated reaction, S-thiolation was higher than oxidation and addition of liposome increased S-thiolation remarkably but showed no effect on oxidation. The effect of liposome on modifications of protein in AAPH and AMVN reaction seemed to be caused by certain reactive degradation products or intermediates of liposome by free radical attack. Peroxidation of liposome was not observed in XOD-initiated reaction. Liposome was gradually peroxidized by AAPH reaction. The peroxidation was inhibited by addition of GSH and phosphorylase. Peroxidation of liposome by AMVN was extreamly fast, and was not affected by GSH and phosphorylase.

• Advances in Traditional Medicine
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• v.10 no.2
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• pp.59-65
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• 2010

In India, Tectona grandis is traditionally used in the treatment of diabetes mellitus and lipid disorder. In the present study, the antihyperglycemic and lipid lowering effect of ethanolic extract of bark of Tectona grandis (TG) was evaluated using alloxan induced diabetes in rats. Alloxan was given at the dose of 140 mg/kg, i.p. After induction of diabetes, TG was administered for 42 days p. o. and simultaneously different biochemical parameters like plasma glucose, liver glycogen content, serum triglyceride, cholesterol, LDL-cholesterol and HDL-cholesterol were estimated. Diabetic control showed significant increase (P < 0.01) in plasma glucose, serum triglyceride, cholesterol, LDL-cholesterol and significant decrease (P < 0.01) in serum HDL-cholesterol and liver glycogen content. Treatment with TG showed significant reduction (P < 0.01) in plasma glucose when compared with diabetic control. The elevated levels of serum triglyceride and cholesterol levels were significantly reduced (P < 0.01) by TG. TG treatment for 42 days showed significant decrease in serum LDL-cholesterol (P < 0.01) and significant increase in serum HDL-cholesterol level (P < 0.01). Moreover, diabetic control there was significant decrease in liver glycogen content which was significantly increased (P < 0.05) by treatment with TG. Hence, from the result obtained in the present study it can be concluded that Tectona grandis has the potential to treat diabetes condition and associated lipid disorder.

• Preventive Nutrition and Food Science
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• v.7 no.2
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• pp.113-118
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• 2002

Alpha-lipoic acid is a known hypoglycemic agent that may be useful in the treatment of diabetes. The objective of this study was to investigate the fate of glucose in isolated muscles incubated with lipoic acid by determining its direct effects on specific metabolic and signaling pathways. Soleus muscles from healthy rats were incubated with lipoic acid in the absence or presence of insulin. Glucose transport, glycogen synthesis, glucose oxidation and lipid synthesis were determined and affects on major pathways associated with insulin signaling were evaluated. Glucose transport was not significantly altered by the addition of lipoic acid to the incubation medium. However, lipoic acid decreased glycogen synthesis in comparison to controls. Glucose oxidation was moderately increased while de-novo lipid synthesis from glucose was inhibited. Wortmannin repressed insulin stimulation of glucose incorporation into glycogen, an effect that was augmented by the combined treatment of wortmannin and lipoic acid. Basal and insulin-stimulated serine phosphorylation of Akt was not changed by the addition of lipoic acid to the incubation medium. These data show that in this in vitro model, lipoic acid did not significantly affect glucose uptake but dramatically modified pathways of glucose metabolism within muscle tissue.

• Biomolecules & Therapeutics
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• v.4 no.4
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• pp.443-448
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• 1996

The effects of Taheebo on the diabetic-piegnant rats and their fetus was investigated. It has been reported that diabetic condition of the pregnant rats can affect the process of liver formation and damage the respiratory function in the fetus. Therefore we investigated the effects of Taheebo on the prevention of liver damage and respiratory failure in the fetus and those results were compared with that of dexamethasone (DXM). In pregnant rats, streptozotocin(STZ, 45 mg/kg, 0.01 M citrate buffer) was injected into the pregnant rats on the third day of pregnancy. Methanol extracts of Taheebo(500 mg/kg p.o.) was administered once daily during pregnancy. DXM (10 $\mu\textrm{g}$/g i.p.) was injected into the pregnant rats in 16th and 18th days of pregnancy. Body weights were measured and fetal number and abortion rate in pregnancy rats were determined. Lecithin/sphingomyelin ratio in amniotic fluid and malondialdehyde, glycogen, triglyceride, protein and cholesterol levels in the liver homogenate were determined. Also blood glucose level was analyzed. Body weights of maternal rats were increased in the all groups except the DXM group. Fetal number of the Taheebo treated group was similar to the control group, and a significant increase in the body weights of fetus was observed in the STZ treated group and the Taheebo treated group compared with the control group. Blood glucose of fetus produced hypoglycemia in the control group and hyperglycemia in the diabetic-pregnant rats. The protein and cholesterol levels in fetus liver were significantly increased in the DXM treated group compared with the control group. Triglyceride content was significantly increased in all groups compared with the control group. Liver malondialdehyde level of fetus in the STZ treated group was similar to the control group. Glycogen level was significantly increased in the all groups compared with the control group. Methanol extract of Taheebo showed hypoglycemic effect on the pregnant rats. However, we could not observe any hypoglycemic effect on the fetus. There's no difference between the control and Taheebo treated group in terms of the levels of triglyceride, cholesterol, protein and glycogen in the fetus liver. Further study to identify the effect of Taheebo on the fetus is under investigation.

• Korean Journal of Food Science and Technology
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• v.16 no.4
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• pp.398-402
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• 1984

These experiments were conducted to investigate the substrate specificity, the hydrolysis products on the various carbohydrates and the hydrolysis rate on the various raw starches of the two purified glucoamylase produced by Rhizopus oryzae. Both of the glucoamylases hydrolyzed amylose, amylopectin, glycogen, soluble starch, pullulan, maltose, maltotriose, maltotetraose, maltopentaose, maltohexaose, maltoheptaose and maltooctaose, but did not act on ${\alpha}-cyclodextrin$, ${\beta}-cyclodextrin$, raffinose, sucrose and lactose. When the reaction mixture of glucoamylase and polysaccharides were incubated $37^{\circ}C$for 32 hours, glucoamylase I hydrolyzed amylopectin, soluble starch and amyloses completely, but hydrolyzing glycogen up to only about 88%. Glucoamylase II hydrolyzed the previous four polysaccharides up to about 100%. Both of the glucoamylases produced only glucose for various substrates and did not have any ${\alpha}-glucosyl$ transferase activity. Both of the glucoamylases hydrolyzed raw glutinous rice starch almost complety, wheras they acted on raw potato starch, raw green banana starch, raw arrow root starch, raw corn starch, raw yam starch and raw high amylose corn starch weakly. Glucoamylase II hydrolyzed raw starches at the higher rate than glucoamylase I.

• Korean Journal of Microbiology
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• v.6 no.2
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• pp.41-53
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• 1968

Studies on the carbohydrate metabolism of yeast as influenced by gamma-irradiation from cobalt-60 have been carried, then the mechanisms of radiation effect on respiration and fermentation were discussed under considerations of permeable changes of irradiated cell membrane. The cells of baker's yeast (Saccharomyces cerevisiae) which had been gamma-irradiated of 240 k.r. doses for an hour, then were put into aerobic oxidation and anaerobic fermentation without substrate. Total and fractionated carbohydrates of irradiated yeast cells were determined by calorimetric method with anthrone and orcinol reagents, the amounts of total carbohydrate, trehalose, RNA-ribose, PCA-soluble glycogen, alkali-soluble glycogen, acetic acid-soluble glycogen, mannan and glucan were determined according to the course of aerobic oxidation and anaerobic fermentation. It is found that the carbohydrates of irradiated cells leak out and amount of the losses teaches eleven times more than that of control, the volume of losses are seems to be replaced by water, it can be suggested the damage of gamma-irradiation occurs in the site of passive transport of cell membrane. The endogeneous aerobic respiration of irradiated cells are increased much more than control, the synthesis of reserve glycogen, glucan and RNA-ribose promoted much more than control. The anaerobic fermentation of irradiated cells are also increased than that of control, but the breakdown of carbohydrate is less than endogeneous respiration of irradiated cells. The synthetic rate is also less than that of aerobic oxidation. In irradiated yeast cells, trehalose is revealed to be primary substrate for endogeneous carbohydrate metabolism, so it is proved that the enzymic patterns are not changed but the activities of enzymes relating endogeneous respiration and autofermentation is activated. It is to be considerable to distiguish endogeneous respiration and autofermentation from exogeneous respiration and fermentation on irradiation, for membrane permeability changes and loses out carbohydrate by ionizing radiation.

• Journal of the Korean Society of Marine Environment & Safety
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• v.14 no.3
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• pp.183-187
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• 2008

Elevated concentrations of suspended solids in the marine enrironment caused by coastal developments have threatened to the marine ecosystem. Effects of suspended solids on the mortality and the modifications of glycogen levels of abalone, Haliotis discus hannai were studied. Abalone were exposed to suspended solids with concentrations of 0 (control), 1,000, 1,500 and 2,000 mg/ L for 96h. These suspended solids had no effect on the mortality of abalone. Significant decreases in the glycogen content of soft tissues were observed over 1,500 mg/ L concentration of suspended solids. These results suggested that abalone could tolerate a high level of suspended solids. However, it is necessary to observe further the long term effects of suspended solids on the physiological responses of abalone.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.55 no.1
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• pp.73-77
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• 2022

The shelf life of oysters Crassostrea gigas, in two different types of packaging containers, polyethylene (PE) and polyethylene terephthalate (PET), was determined by evaluating the pH, glycogen and soluble protein content, turbidity, and viable cell count. After 7 days of storage, the pH of the packing water in the PE container decreased to 5.88, while the pH in the PET container decreased to 6.03. In the PE container, the glycogen content of the oysters decreased by 0.85 g/100 g and the soluble protein content and turbidity of the packing seawater increased by 1,927.21 mg/100 g and 3.24 McF, respectively. In the PET container, the glycogen content of the oysters decreased by 0.96 g/100 g and the soluble protein content and turbidity of the packing seawater increased by 1,674.75 mg/100 g and 0.98 McF, respectively. The reaction rate constants (K) were as follows: glycogen content, -0.18 (PE) and -0.10 (PET); soluble protein content, 0.29 (PE) and 0.26 (PET); and turbidity, 0.41 (PE) and 0.06 (PET). These results suggested that PET can be used as a new packaging container material for raw oysters because the quality is maintained and it offers more convenient handling during distribution.