• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.650-654
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• 2008

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

• Journal of the Korean Society of Food Science and Nutrition
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• v.32 no.2
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• pp.278-286
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• 2003

Alcohol is well known agent which can damage the human tissues such as liver via stimulating lipid peroxidation. On the other hand, carotenoids in addition to vitamins A, C and I play important roles in protecting these oxidative damages as well as preventing the production of free radicals. This study was carried out to investigate the effect of dietary vitamin A on lipid peroxidation and antioxidants status in ethanol-treated rats. In the experiment, male Sprague-Dawley rats weighing 160~180 g were given a liquid diet containing 36% of total calories as ethanol for 7 weeks. The pair-fed control rats received an isocaloric amount of diet containing sucrose instead of ethanol on the following day Additionally, the liquid diet contained adequate amount of $\beta$-carotene, retinyl acetate or 13-sis-reinoic acid except vitamin A-deficient diet. The results obtained are as follows. The levels of plasma and hepatic lipid peroxide were increased after chronic ethanol feeding in rats. Retinyl acetate supplementation significantly reduced lipid peroxidation induced by ethanol feeding Glucose 6-phosphatase activity was significantly reduced in rats fed vitamin A-deficient diet with ethanol and alkaline phosphatase activity was significantly induced in rats fed 13-cis-reinoic acid diet with ethanol. Catalase and alcohol dehydrogenase activities did not show a consistent tendency in experiment groups. The hepatic antioxidant enzyme activities did not significantly changed by chronic ethanol feeding groups. The striking decrease in conversion of $\beta$-carotene to retinol was observed in rats fed a $\beta$-carotene diet with ethanol feeding The level of retinol and retinoic acid in plasma and liver was decreased after chronic ethanol administration Based on this result, these data suggest that ethanol feeding enhances oxidative stress especially in those fed a vitamin A-deficient diet, and vitamin A supplementation, especially, retinyl acetate intake can prevent enhanced lipid peroxidation and related damage to some extent.

• Journal of Life Science
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• v.20 no.7
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• pp.1012-1018
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• 2010

The aim of this study was to determine whether Alzheimer's amyloid precursor protein (APP) is dysregulated in adipose tissues of C57BL/6 male mice by high-fat diet (HFD) induced obesity, aging, or streptozotocin (STZ)-induced diabetes. APP mRNA expression was examined by quantitative real-time PCR (QPCR) in subcutaneous (SAT) and epididymal adipose tissues (EAT) from mice in 8 different condition groups. By combining conditions of age (16 weeks/26 weeks of age), diet (normal diet (ND)/high-fat diet), and induction of diabetes (non-diabetic/diabetic), 88 mice were divided into 8 different groups. QPCR demonstrated that APP expression in SAT was significantly increased by about two-fold in HFD-induced obese mice compared to both 16 week-old and 26 week-old mice in the ND group (16 weeks p=0.001; 26 weeks p<0.0001), but no changes in EAT was found. Particular effects of aging on APP gene expression were not observed in either adipose tissue depots. Significantly decreased APP expression was found in SAT in STZ-induced diabetic mice fed on ND or HFD at 16 weeks of age (ND p<0.05; HFD p<0.01). Linear regression analysis demonstrated that APP expression levels correlated with body weight in both the non-diabetic group (R=0.657, p<0.0001, n=39) and the diabetic group (R=0.508, p=<0.0001, n=49), but did not correlate with plasma glucose levels, which suggests that decreased APP expression in STZ-induced diabetic mice is most likely due to weight loss rather than hyperglycemia. These data confirm APP dysregulation by weight changes in humans and suggest a possible role linking midlife obesity with the later development of amyloidogenesis in the brain of older patients with Alzheimer's disease.

• Journal of Mushroom
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• v.16 no.2
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• pp.118-124
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• 2018

In this study, we aimed to investigate the effects of dietary supplementation with fruiting body of Agaricus brasiliensis (AB) mushroom on the lipid profiles of serum and histological patterns of liver of high cholesterol-fed rats. Five-week-old, female Sprague-Dawley albino rats were divided into three groups of 8 rats each, including a normal control-diet (NC) group, a high-cholesterol diet (HC) group, and a group fed high-cholesterol diet supplemented with 5 % powder of Agaricus brasiliensis fruiting bodies (HC+AB). Total serum cholesterol, low density lipoprotein (LDL), and triglyceride (TG) concentrations in the HC+AB group were significantly reduced when compared with those in the HC group. Body weight in the HC+AB group was significantly lower than that in the HC group, whereas no adverse effects were observed on the levels of plasma albumin, creatinine, blood urea nitrogen, uric acid, glucose, and total protein. In the HC+AB group, liver enzyme activities related to liver function, such as GOT and GPT, presented values lower than those in the HC group and were very similar to the ones in the NC group. Excretion of total lipid and cholesterol in feces in the HC+AB group was significantly higher than that in the NC and HC groups, indicating that mushroom feeding inhibits the absorption of lipid cholesterol in the intestine. Liver histopathological analyses revealed that rats fed with HC diet developed fat liver disease, whereas only small amounts of fat were deposited in the livers of the HC+AB group. In conclusion, the results suggest that fruiting body powder of A. brasiliensis provides health benefits to high-cholesterol-fed rats by lowering body weight and the risk of atherogenic lipid profile.

• Journal of Life Science
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• v.20 no.12
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• pp.1812-1819
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• 2010

Skipping breakfast is a risk factor closely related to metabolic syndrome and obesity. We analyzed the relationship between breakfast size, metabolic syndrome and obesity. The study included 5,548 adults who visited a health promotion center at Pusan National University from January to November of 2006. Subjects were divided into four groups according to breakfast size - skipper group (no breakfast), small intake group, medium intake group and large intake group. 959 (17.3%) of the 5548 subjects were included in the Skipper group. Intake of daily calories, proteins, carbohydrates and fats was the lowest in the Skipper group. Breakfast size increased linearly with an increased intake of daily calories, proteins, carbohydrates and fats. Body mass index ($23.4\;kg/m^2$) and waist circumference (79.6 cm) were the lowest in the Small intake group. In the Small intake group, triglycerides, fasting plasma glucose, systolic blood pressure and diastolic blood pressure were the lowest, and high density lipoprotein cholesterol levels were the highest. The number of metabolic risk factors was the lowest in Small intake group. Odds ratio of metabolic syndrome (Odds ratio=0.612) was the lowest in Small intake group. Along with increasing breakfast size, the odds ratio also increased. In this study, breakfast size was found to influence metabolic risk factors. Skipping breakfast worsened metabolic risk factors, while a small breakfast size had a favorable effect on metabolic risk factors.

• Korean journal of food and cookery science
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• v.29 no.2
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• pp.185-192
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• 2013

The present study was conducted to evaluate the effects of Agarum cribrosum on weight change and defecation states in rats with constipation induced by loperamide. Food intake and body weight both decreased in the 5% Agarum cribrosum and loperamide-treated group (SD5) and 10% Agarum cribrosum and loperamide-treated group (SD10), whereas fecal water contents increased by 1.6 and 2.1-fold in the SD5 and SD10 groups, respectively. The concentrations of total-cholesterol, HDL-cholesterol and triglyceride in the sera of the SD5 and SD10 groups were lower than those in the control (C) group. However, the biochemical parameters, GOT (glutamic oxaloacetic transaminase), GPT (glutamic pyruvic transaminase), and glucose levels, were not affected by the level of Agarum cribrosum. In addition, the concentrations of total-cholesterol and triglyceride in the livers of the SD5 and SD10 groups were also lower than those in the control group. The results of the present study demonstrated that Agarum cribrosum might ameliorate constipation symptoms, and lower lipid concentrations in the blood and liver.