• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.2
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• pp.213-217
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• 2001

Purpose: Glycogen storage disease type Ia (GSD Ia) is an autosomal recessive disorder of glycogen metabolism caused by glucose-6-phosphatase (G6Pase) deficiency. The clinical manifestations of G6Pase deficiency include growth retardation, hepatomegaly, hypoglycemia, lactic acidemia, hyperlipidemia and hyperuricemia. Many mutations of this gene have been found worldwide in various ethnic groups, establishing the molecular basis of GSD Ia. To elucidate a spectrum of the G6Pase gene mutations in Korean, we analyzed mutations in Korean patients with GSD Ia. Methods: Both alleles of 9 unrelated GSD 1a patients were studied by PCR and direct DNA sequencing methods. In all patients, GSD 1a was diagnosed by the enzyme assay for the liver biopsy specimen. Results: In Korean, the most prevalent mutation was g727t substitution in exon 5, which has been reported to cause abnormal mRNA splicing: Sixteen out of 18 alleles were found to have this mutation. In addition, we identified one novel mutation, a c611g, converting a proline to an alanine at codon 178. Conclusion: Our findings suggest that a screening for the g727t mutation by noninvasive molecular method can detect most cases of GSD Ia in Korean patients.

• Journal of the Korean Applied Science and Technology
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• v.37 no.4
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• pp.682-687
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• 2020

This study was performed to investigate the antidiabetic effect of ethanol extract of Phelladrindron Amurense Rupr (P.A) in Streptozotocin(STZ) induced diabetic rats. Diabetes was induced by intravenous injection of STZ at a dose of 45mg/kg,b.w. dissolved in citrate buffer. The ethanol extract of P. A was orally administrated once a day for 7 days at a dose of 1,000mg/kg. The content of serum glucose, was significantly decreased in P.A treated group compared to the those of STZ-control group. The content of hepatic glycogen and activities of glucokinase(GK) and glucose-6-phosphate dehydrogenase(G-6-PDH) were significantly increased(p<0.05), and activity of glucose-6-phoshatase(G-6-Pase) was significantly decreased(p<0.05) in P.A treated group compared to those of STZ-control group, These results indicated that ethanol extract of P.A have antidiabetic effect in STZ-induced diabetic rats.

• Clinical and Experimental Pediatrics
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• v.48 no.8
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• pp.877-880
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• 2005

Purpose : Glycogen storage disease type Ia(GSD Ia) is an autosomal recessive disorder caused by the deficiency of glucose-6-phosphatase(G6Pase). The aim of the study was to investigate the spectrum of G6Pase gene mutations and relationship between genotype and clinical findings in Korean patients with GSD Ia. Methods : Genomic DNA was extracted from peripheral leukocytes of 20 patients with GSD Ia. The five exons of G6Pase gene were amplified and PCR products were directly sequenced. The frequency of short stature, hypoglycemia, hypercholesterolemia, hyperuricemia, hypercalciuria, nephrocalcinosis and hepatic adenoma was compared between 727G>T homozygotes and 727G>T compound heterozygotes. Results : A total of 5 different mutations were identified. The most common mutation was the 727G>T with an allele frequency of 80%. All patients were either homozygous(12/20) or heterozygous(8/20) for the 727G>T mutation. G122D was found in 3 patients, P178A in 1, G222R in 2, and S339R in 2. There was no difference in the frequency of short stature, hypoglycemia, hypercholesterolemia, hyperuricemia, nephrocalcinosis, and hepatic adenoma between 727G>T homozygotes and heterozygotes. Conclusion : Diagnosis of GSD Ia can be based on clinical and biochemical abnormalities combined with mutation analysis instead of enzymatic diagnosis that requires liver biopsy. Homozygosity for the 727G>T does not seem to alter the disease phenotype as compared with the heterozygous state.

• Journal of Nutrition and Health
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• v.23 no.6
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• pp.418-426
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• 1990

This study examines the effects of choline deficiency and 2-acetylaminofluorene(2-AAF) on the lipid peroxide values, glucose 6-phosphatase(G6Pase) and glutathione S-transferase (GST) activities in rats fed different dietary fats. Weanling Sprague Dawley male rats fed the diets containing 15% beef tallow or 15% corn oil with vitamin fortification mixture or choline free vitamin mixture for 10 weeks. At 3th and 5th week, 2-AAF was injected twice each week intraperitoneally. Total 2-AAF injection was four times. 2-AAF and choline deficiency increased lipid peroxidation in corn oil groups, so the role of 2-AAF and choline deficiency in lipid peroxidation was more important in corn oil groups than beef tallow groups. G6Pase activities tended to be decreased by 2-AAF in choline deficient groups, and in corn oil groups, the enzyme activities were decreased significantly in all subgroups compaired to beef tallow groups. GST activities were increased by 2-AAF in beef tallow groups and choline deficiency in corn oil groups, and might defence against carcinogen metabolism and lipid peroxidation.

• Preventive Nutrition and Food Science
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• v.8 no.2
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• pp.158-161
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• 2003

This study investigated the effects of a ${\gamma}$-irradiated pork (0-30 kGy) diet on lipid peroxidation, cytochrome P-450 content, microsomal glucose 6-phosphatase (G-6-Pase) activity and antioxidative defense systems in diethylnitrosamine (DEN)-induced rat hepatocarcinogenesis. The body weight of rats fed irradiated diets did not change significantly. Liver weight was significantly increased by the administration of DEN, but not by irradiated diets at any dose level. There were no significant effects of gamma irradiation on the content of microsomal malondialdehyde (MDA), cytochrome P-450, or on the activity of G-6-Pase. However, with DEN treatment, cytochrome P-450 content was significantly increased while microsomal G-6-Pase activity was significantly decreased. The ${\gamma}$-irradiated diet supplement did not affect serum retinol or $\alpha$-tocopherol concentrations. However, it did cause a significant decrease in hepatic retinol at 30 kGy. With DEN treatment, hepatic retinol content was even more significantly (p<0.05) decreased compared to the non-irradiated control. The enzyme activities related to antioxidative defense systems, including glutathione peroxidase (GSH-Px), glutathione reductase (GSH-Rx) and glutathione S-transferase (GST) were not affected by gamma irradiation. Those results suggest that an irradiated pork diet up to 30 kGy may not cause a health hazard in experimental animals.

• Journal of the Korean Applied Science and Technology
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• v.32 no.2
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• pp.226-231
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• 2015

This study was carried to investigate the antidiabetic effect of ethanol extract in Streptozotocin(STZ)-induced diabetic rats. Diabetes was induced by intravenous injection of STZ at a dose of 45mg/kg dissolved in citrate buffer. The ethanol extract of Forsythia Koreana(F.K) was orally administrated once a day for 7 days at a dose of 1,000mg/kg. The contents of serum glucose, triglyceride(TG), total cholesterol were significantly decreased in F.K treated group compared to the those of STZ-control group. The content of hepatic glycogen and activity of glucokinase(GK) were significantly increased, and activity of glucose-6-phoshatase(G-6-Pase) was significantly decreased in F.K treated group compared to the those of STZ-control group, but activity of glucose-6-phosphate dehydrogenase(G-6-PDH) was not significantly increased, These results indicated that ethanol extract of F.K would have antidiabetic effect in STZ-induced diabetic rats.

• Journal of the Korean Applied Science and Technology
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• v.36 no.3
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• pp.898-904
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• 2019

This study was carried to investigate the antidiabetic effect of ethanol extract of Astragali Radix(A.R) in Streptozotocin(STZ) induced diabetic rats. Diabetes was induced by intravenous injection of STZ at a dose of 45mg/kg dissolved in citrate buffer. The ethanol extract of A. R was orally administrated once a day for 7 days at a dose of 1,000mg/kg. The contents of serum glucose, triglyceride(TG), total cholesterol were significantly decreased in A.R treated group compared to the those of STZ-control group. The content of hepatic glycogen and activities of glucokinase(GK) and glucose-6-phosphate dehydrogenase(G-6-PDH) were significantly increased, and activity of glucose-6-phoshatase(G-6-Pase) was significantly decreased in A.R treated group compared to the those of STZ-control group, These results indicated that ethanol extract of A.R would have antidiabetic effect in STZ-induced diabetic rats.

• Journal of Korean Academy of Nursing
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• v.19 no.3
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• pp.299-306
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• 1989

In order to evaluate the effect of fish oil on lipid drogenase(G6PDH), malic enzyme(ME), glucose-6-phosphatase(G6Pase) activities were measured in liver and adipose tissue of rats fed 13 days supplemented fish oil at the level of 10% (W/W). Two other groups of rats were fed 10% soybean oil or lard to compare with the effect of fish oil. In all groups, activities of hepatic G6PDH and ME were depressed from the beginning of feeding. This effect was greatest (50%) in fish oil group. Hepatic G6Pase was highest in rats fed lard. When the level of fish oil was reduced to half, as total fat content was maintained at the level of 10% by complementary lard, lipogenic enzyme depressing effect of fish oil was as significant as shown in 10% fish oil diet. Hepatic G6PDH was depressed significantly(14%) in rats fed fish oil as low as 2％. On the other hand, changes in adipose tissue G6PDH and ME activities were small. Adipose tissue G6Page activity increased slightly in rats fed with increasing fish oil(above 0.5%). It is suggested that fish oil alter, more markedly than either soybean oil or lard, cellular lipid metabolism by reducing activities of hepatic lipogenic enzymes.

• Biomedical Science Letters
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• v.6 no.4
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• pp.245-251
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• 2000

In order to search the target organ of cylclohexane toxicity, the rats were intraperitoneally treated with cyclohexane (1.56 g/kg of body wt.) four times every other day. In the increasing rate of organ weight per body weight (%) in cyclohexane-treated animals, the lung was highest among the liver, spleen, small intestine, stomach, heart and kidney. And in the decreasing rate of glucose-6-phosphatase (G-6-Pase) activity in each organ, that of lung was also highest among all organs. Lung MDA content was significantly increased (p<0.05) by the cyclohexane treatment. On the other hand, microsomal aniline hydroxylase activity in lung tissue both of control and cyclohexane-treated rats was greatly low as could be scarcely measured, but that in liver possessing high activity was significantly increased (p<0.05) in cyclohexane-treated rats compared with control. Alcohol dehydrogenase activity in lung was markedly higher than that of liver and the latter was significantly (p<0.05) increased by the cyclohexane treatment. In conclusion, cyclohexane treatment to the rats showed mainly lung toxicity and it may be responsible for cyclohexanon, cyclohexane metabolite, distributed from liver.

• Journal of the Korean Applied Science and Technology
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• v.34 no.2
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• pp.254-259
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• 2017

The ethanol extraction yield of Liriope platyphylla(Lp) was about 30.7% by extract apparatus. This study was done to investigate the carbohydrate metabolism related enzyme activities effects of Lp in streptozotocin (STZ)-induced diabetic rats. The contents of serum glucose, triglyceride (TG) were significantly decreaed in Lp treated group compared to the those of STZ-control group, also content of Total cholesterol was decreased. High density lipoprotein (HDL)-cholesterol was increased in Lp treated group. The activity of glucose-6-pase(G-6-Pase) was significantly decreased in Lp treated group. Also the activity of glucokinase(Gk) was increaed in Lp treated group. The content of hepatic glycogen was significantly increaed in Lp treated group. These results indicated that ethanol extract of Lp would have antidiabetic effect in STZ-induced diabetic rats.