• Journal of Genetic Medicine
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• 제4권1호
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• Journal of Integrative Natural Science
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• 제8권1호
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• pp.40-47
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• 2015

Glycogen synthase kinase 3 (GSK-3) is a serine/threonine protein kinase that has recently emerged as a promising target in drug discovery. It is involved in multiple cellular processes and associated with the pathogenesis of several diseases. A three-dimensional quantitative structure-activity relationship (3D-QSAR) analysis was performed on a series of GSK-3 inhibitors to understand the structural basis for inhibitory activity. Comparative molecular field analysis (CoMFA) method was used to derive 3D-QSAR models. A reliable CoMFA model was developed using ligand-based alignment scheme. The model produced statistically acceptable results with a cross-validated correlation coefficient ($q^2$) of 0.594 and a non-cross-validated correlation coefficient ($r^2$) of 0.943. Robustness of the model was checked by bootstrapping and progressive scrambling analysis. This study could assist in the design of novel compounds with enhanced GSK-3 inhibitory activity.

• The Korean Journal of Pharmacology
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• 제17권1호
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• pp.33-39
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• 1981

In this paper, the influences of adrenergic neuronal blockades of different mode: guanethidine and ${\alpha}$-methyl-para-tyrosine on the changes induced by carbon tetrachloride $(CCl_4)$ of hepatic total lipid, glycogen, and lipid peroxide contents and serum lactic dehydrogenase activity were investigated in male mice. The results obtained were summarized as follows: 1) The hepatic total lipid and lipid peroxide contents and serum lactic dehydrogenase activity were markedly increased by $CCl_4$, but hepatic glycogen content were decreased. 2) The hepatic total lipid and lipid peroxide contents and serum lactic dehydrogenase activity were not significantly changed by guanethidine(20mg/kg) or ${\alpha}$-methyl-para-tyrosine (5 mg/kg) injection. 3) The increase of hepatic total lipid induced by $CCl_4$ was inhibited by the pretreatment of guanethidine or ${\alpha}$-methyl-para-tyrosine, and the increase of hepatic lipid peroxide content induced by $CCl_4$ was slightly inhibited by them. But the decrease of hepatic glycogen content and the increase of serum lactic dehydrogenase activity induced by $CCl_4$ were not affected by them.

• The Korean Journal of Pharmacology
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• 제16권2호
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• pp.31-38
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• 1980

Calvert et al. formulated the hypothesis that carbon tetrachloride ($CCl_4$) acted on the central nervous system to produce and intensify sympathetic discharge which resulted in anoxic necrosis of the liver. Recknagel suggested that the essential feature of $CCl_4$ hepatotoxicity depended on the cleavage of it to $CCl_3$(free radical) and the peroxidative decomposition of cytoplasmic membrane structural lipids. And there are many reports which show the increase of adrenergic activity in hyperthyroidism. In this paper, the influence of thyroxine on the hepatotexicity of carbon tetrachloride was investigated in mice. The results obtained were summarized as follows; 1) Hepatic total lipid and lipid peroxide contents were slightly decreased by L-sodium thyroxine injection(4mg/kg/day for 4days or 6days), but hepatic glycogen content was significantly decreased. 2) Hepatic total lipid and lipid peroxide contents and serum lactic dehydrogenase activity were significantly increased by $CCl_4$ (4 ml/kg single dose or triple dose: 4ml/kg/day for 3days), but hepatic glycogen content was significantly decreased. 3) The increase of hepatic total lipid and lipid peroxide contents and serum lactic dehydrogenase activity induced by $CCl_4$ were significantly inhitited by the pretreatment of thyroxine. 4) The decrease of hepatic glycogen induced by $CCl_4$ was not affected by the pretreatment of thyroxine.

• Clinical and Experimental Pediatrics
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• 제52권12호
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• pp.1383-1387
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• 2009

We report a case of an 18-month-old girl with glycogen storage disease type Ib (GSD Ib). Her neutrophil counts had gradually decreased to less than $500/{\mu}L$ by the age of 3 years. However, there were no recurrent bacterial infections. Mutation analysis of the glucose-6-phosphate translocase (G6PT) gene revealed a compound heterozygous missense mutation (Ala148Val/Gly273Asp).

• The Korean Journal of Pharmacology
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• 제18권1호
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• pp.33-41
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• 1982

In this paper, the influences of adrenergic blockades; propranolol and phenoxybenzamine on the changes of hyperglycemic action, hepatic glycogen content, and brain norepinephrine (NE) content induced by clonidine were investigated in the male mice. The results obtained were summarized as follows: 1) Blood glucose level was significantly increased by clonidine $(30{\mu}g/kg)$. The increase of blood glucose level induced by clonidine was not affect by the propranolol (10mg/kg) pretreatment, but significantly inhibited by the phenoxybenzamine (10mg/kg) pretreatment. 2) Hepatic glycogen content was moderately inhibited by clonidine. The decrease of hepatic glycogen content induced by clonidine was not affected by the propranolol and phenoxybenzamine pretreatment. 3) Brain NE content was significantly increased in 30 minutes and 60 minutes after clonidine treatment. The increase of brain NE content induced by clonidine was significantly inhibited by the phenoxybenzamine pretreatment. The increase of train NE content induced in 90 minutes and 120 minutes after clonidine treatment was more markedly increased by the propranolol pretreatment.

• Asian-Australasian Journal of Animal Sciences
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• 제11권3호
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• pp.300-306
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• 1998

An experiment was conducted to investigate correlations of litter size and average serum progesterone concentrations during pregnancy with mammary gland growth and development at parturition. Twenty ewes (5, 9, 4, and 2 ewes carrying 0, 1, 2, and 3 lambs, respectively) were used to measure weekly serum progesterone concentration during pregnancy. At parturition, the experimental ewes were slaughtered for determination of mammary gland growth and development at parturition (mammary dry fat-free tissue [DFFT], DNA, RNA, collagen, protein, and glycogen). Correlation of mammary DFFT with litter size and averages serum progesterone concentrations were 0.75 and 0.72, respectively. Litter size or maternal serum progesterone concentrations did not correlate with the mammary DNA concentration. However, litter size or maternal serum progesterone concentrations positively correlated (p < 0.01) with the mammary RNA and protein concentrations, but negatively correlated with the mammary collagen (p < 0.01) and. glycogen (p < 0.05) concentrations. Litter size or maternal serum progesterone positively correlated (p < 0.01) with the total mammary DNA, RNA, collagen, protein and glycogen contents. These results implied that the increased concentrations of progesterone with the increased litter size during pregnancy improved mammary gland growth and development at parturition.

• Korean Journal of Fisheries and Aquatic Sciences
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• 제42권5호
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• pp.481-486
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• 2009

In 2007, 43.5% mortality of the cultured oyster population occurred in Gamak Bay. Mortality rapidly increase in September and peak in October. To prevent future mass-mortality event, we investigated spawning and variation of oyster body composition. The main spawning period of culture oyster occurred from August to September. Condition index and body composition (protein and glycogen) appeared to be influenced by the spawning activity. Condition index and glycogen content in September were lowest (13.5% and 5.6 mg/g, respectively). However, protein, lipid and glycogen contents did not rapidly recover after the spawning activity. The data indicates that mass-mortality of cultured oysters in Gamak Bay may be due to deteriorated health, spawning activity, stress of the high water temperature and decreasing food resources.

• Journal of Sericultural and Entomological Science
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• 제38권2호
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• pp.118-123
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• 1996

Dietary supplementation of folic acid to silkworm larvae in different concentration (100, 200 and 300 ${\mu}$g/ml) to the 4th and the 5th instar resulted in a significant increase in economic parameters, such as female and male cocoon weights, shell weights, egg productivity and egg hatching percentage. However, larval duration was significantly decreased, as compared with that of carrier control. The increase in the glycogen and protein contents of the fat body was not significant, whereas the increase in trehalose content of haemolymph in all the treated groups and protein content of fat body and haemolymph in 100 ${\mu}$g/ml treated groups were significant. However, the haemolymph protein was significantly decreased in 300${\mu}$g/ml treated group.

• Journal of Nutrition and Health
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• 제36권10호
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• pp.981-989
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• 2003

The hypoglycemic effects of four edible plants (Angelicae tenuissimae (A. ten.), Pleurospermum kamtschaticum (P. kam.), Adenophora remotiflora (A. rem.) and Zanthoxylum schinifolium (Z. sch.)) in streptozotocin (STZ) -induced diabetic rats were investigated. Sprague-Dawley male rats weighing 190-230 g were induced diabetes mellitus by the STZ injection (45 mg/kg) into the tail vein and were divided into six groups ; normal, STZ-control and four edible plant groups (A. ten., P kam., A. rem. and Z. sch. groups). Normal and STZ-control groups were fed a AIN-93 diet and four groups of STZ-induced diabetic rats were fed one of each experimental diets containing 10％ of the edible plant powder for 4 weeks. Diabetic rats showed the lower weight gain compared to the normal rats. In experimental groups except P. kam., AST activities were close to normal. A. ten. group were lowered ALT activities slightly. The plasma glucose levels of the diabetic experimental groups were significantly decreased at 4th week. The plasma insulin levels in diabetic experimental groups were not significantly different compared to the STZ-control group. The liver glycogen levels in STZ injected rats were significantly lower in compared to the normal rats. However no significant differences were found in response experimental plants intake in diabetic rats. The muscle glycogen were not significantly different among all the groups.