• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.13 no.1
• /
• pp.30-36
• /
• 2013

6-pyruvoyltetrahydropterin synthase (PTPS) deficiency is autosomal recessive disorder and the most common type of tetrahydrobiopterin (BH4) deficiency. It is caused by deficiency of PTPS, a cofactor involved in the biosynthesis of BH4 from guanosine triphosphate (GTP). Unlike classical phenylketonuria, which needs restriction of dietary phenylalanine for whole life, BH4 deficiency is treated by tetrahydrobiopterin, levodopa, and 5-hydroxytryptophan replacement. So it is important to make accurate diagnosis and initiate treatment as soon as possible for a better prognosis. There is no retrospective study of Korean patients undergoing long-term treatment for PTPS deficiency. We report 9 Korean patients with PTPS deficiency and their laboratory findings including BH4 loading tests, urine pterin tests, genotypes, dihydropteridine reductase (DHPR) activities and clinical manifestations including medication and developmental delay existence.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.5 no.1
• /
• pp.9-17
• /
• 2005

Homocystinuria is a metabolic disorder caused by a deficiency of cystathionine ${\beta}$-synthase (CBS). Patients with homocystinuria show clinical symptoms such as mental retardation, lens dislocation, vascular disease with life-threatening thromboembolisms and skeletal deformities. Generally, the major treatments for CBS deficiency include pharmacologic doses of pyridoxine or dietary restriction of methionine. However, there is no effective treatment for this disease up till today and gene therapy can be an attractive novel approach to treatment of the disease. We investigated whether a recombinant adeno-associated virus could be used as a CBS gene transfer vector to reduce the excessive homocysteine level in the homocystinuria mouse model. Recombinant adeno-associated virus vector encoding the human CBS gene (rAAV-hCBS), driven by EF1-a promoter, was infused into CBS-deficient mice ($CBS^{-/-}$) via intramuscular (IM) and intraperitoneal (IP) injection. IP injection was more efficient than IM injection for prolongation of lives and reduction of plasma homocysteine levels. After 2 weeks of gene transfer by IP injection, serum homocysteine level was significantly decreased in treated mice compared with the age-matched controls and the life span was extended about 1.5 times. Also, increased expression of CBS gene was observed by immunohistochemical staining in livers of treated $CBS^{-/-}$ mice and microvesicular lipid droplets was decreased in cytoplasm of liver. These results demonstrate the possibility and efficacy of gene therapy by AAV gene transfer in homocystinuria mice.

• Korean Journal of Poultry Science
• /
• v.29 no.1
• /
• pp.25-35
• /
• 2002

Two experiments were conducted to studs the effects of early-life feed restriction, ages and duration on compensatory growth, feed efficiency, and abdominal fat pad deposition in broilers. The chicks were randomly assigned to four treatments (experiment 1) or seven treatments (experiment 2) . Each dietary treatment was replicated with three floor pens in both experiments, and twenty chicks were assigned to each pen. In experiment 1, birds in control group (C) were full-fed throughout the study. For treatments 1, 2, and 3, bards were fed diets containing 0.75 kcal ME/gB $W^{o.67}$/d for 3 d(T1), 1.5 ㎉ ME/gB $W^{o.67}$/d for 5 d (T2), and 2.25 local ME/gB $W^{0.67}$/d for 7 d (T3), all imposed beginning at 7 d of age. The selected value seems appropriate for maintenance feeding because the feed restriction at T1 resulted no growth in virtually. Although the birds previously subjected with restricted feeding significantly (P<0.05) enhanced growth partly (in 22~49 d), the weight gain over the entire growth period (7-49 d) was not influenced by the restricted feeding Programs. Whereas, the restricted feedings had effects with significant (P<0.75) increase in feed intake not only in 22~49 d, but in overall period (7-49 d). The feed conversion for the birds previously subjected with restricted feeding was significantly (P<0.05) improved after 21 d. The birds with restricted feedings (T1,T2,T3) showed better (P<0.05) feed conversion ratio than C both in 22-49 d and in 7-49 d. The restricted feeding programs did not show effects in reducing for abdominal fat pad deposition or mortality In experiment 2, the birds of C were full-fed in overall period. But for treatments 1, 2, 3, 4, 5, and 6 (T1-T6),the birds were fed diets containing 0.75 ㎉ ME/gB $W^{0.67}$/d for 3 or 5 d, starting at either 4, 7 or 10 d of age. The selected value seems appropriate for maintenance feeding because the feed restriction resulted no growth in virtually; which was same as experiment.

• Journal of Korean Society of Forest Science
• /
• v.104 no.1
• /
• pp.67-75
• /
• 2015

This research is to investigate the four years growth mountain-cultivated ginseng ripened twenty-two weeks into four years fermented persimmon vinegar (tentatively: Sansamcho) ingestion on obese-related factors during dietary control. The Sansamcho was ingested orally, two times a day, after every meal for six weeks to the male rats. Groups were divided into the control (CON), the restricted diet (RD), and the weight cycling (WC). And, each groups has its own sub-groups as the -control (-CON), 2.5 times diluted Sansamcho ingestion (-MPV2.5), and 5.0 times diluted Sansamcho ingestion (-MPV5.0) groups, respectively. The number of rat was consisted of seven in each group. After six weeks rearing periods was done, abdominal fats (retroperitoneal fat, mesentery fat, and epididymal fat) and energy metabolic-related protein (AMPK: AMP-activated protein kinase; PPAR-${\alpha}$: peroxisome proliferator-activated receptor-${\alpha}$; and CPT-1: carnitine palmitoyltransferase-1) were weighed and analyzed. Amount of stored fat was significantly or tended to decrease by Sansamcho ingestion. In addition, sum of fats increasing were suppressed by the material. On the contrary, energy metabolism-related protein expression was significantly increased or tended to increase by Sansamcho ingestion. This results suggested that increased energy metabolism using Sansamcho was restrained effectively visceral fat store by high-fat diet and/or dietary control. In other words, it has a good function to suppress weight cycling which is the most insoluble problem. Therefore, the fusion material, Sansamcho, may expect to utilize as the obese-suppression-food.

• Asian-Australasian Journal of Animal Sciences
• /
• v.27 no.12
• /
• pp.1695-1704
• /
• 2014

Maternal malnutrition during pregnancy may give rise to female offspring with disrupted ovary functions in adult age. Neonatal ovary development predisposes adult ovary function, yet the effect of maternal nutrition on the neonatal ovary has not been described. Therefore, here we show the impact of maternal protein restriction on the expression of folliculogenic and steroidogenic genes, their regulatory microRNAs and promoter DNA methylation in the ovary of neonatal piglets. Sows were fed either standard-protein (SP, 15% crude protein) or low-protein (LP, 7.5% crude protein) diets throughout gestation. Female piglets born to LP sows showed significantly decreased ovary weight relative to body weight (p<0.05) at birth, which was accompanied with an increased serum estradiol level (p<0.05). The LP piglets demonstrated higher ratio of bcl-2 associated X protein/B cell lymphoma/leukemia-2 mRNA (p<0.01), which was associated with up-regulated mRNA expression of bone morphogenic protein 4 (BMP4) (p<0.05) and proliferating cell nuclear antigen (PCNA) (p<0.05). The steroidogenic gene, cytochrome P450 aromatase (CYP19A1) was significantly down-regulated (p<0.05) in LP piglets. The alterations in ovarian gene expression were associated with a significant down-regulation of follicle-stimulating hormone receptor mRNA expression (p<0.05) in LP piglets. Moreover, three microRNAs, including miR-423-5p targeting both CYP19A1 and PCNA, miR-378 targeting CYP19A1 and miR-210 targeting BMP4, were significantly down-regulated (p<0.05) in the ovary of LP piglets. These results suggest that microRNAs are involved in mediating the effect of maternal protein restriction on ovarian function through regulating the expression of folliculogenic and steroidogenic genes in newborn piglets.

• Journal of the Korean Society of Food Science and Nutrition
• /
• v.34 no.5
• /
• pp.644-651
• /
• 2005

Dietary supplement mainly made of herb and fiber was examined whether it could reduce body weight and fat, modify blood lipid concentrations and bowel habits in 30 collegians without intentional diet restriction or lifestyle change for 5 weeks. Free-living subjects were required to take diet pills 2 times daily 30 minutes before meals. Before the study began, 24 hr recall diet record and the questionnaires had been collected. Anthropometric measurements (height, weight, waist and hip circumferences, triceps and abdomen skinfold thickness, and body fat) were performed and blood samples were withdrawn before and after the study. Blood lipid fractions analyzed were total cholesterol (TC), triglyceride (TG), HDL-cholesterol and LDL-cholesterol. After the trial, body weight, body mass index, and percent ideal body weight of the subjects were reduced to mean of 0.5 kg, 0.2 and $0.9\%$, respectively (p<0.05). Percent body fat, triceps and abdomen skinfold thickness, and waist and hip circumferences were all reduced significantly except for $\%$ abdominal fat, but $\%$ body muscle mass increased from $36.5\%$ to $37.4\%$ (p=0.000). TC and TG were remarkably diminished (p<0.01) and LDL-cholesterol tended to decrease, but no change was observed in HDL-cholesterol. Bowel movements were also increased (p<0.01). In conclusion, this specific herb and fiber-rich dietary supplement reduced body weight and body fat indices, improved anthropometric indices, modified blood lipid fractions and bowel movement desirably. The study suggest that herb and fiber-rich dietary supplement might help control body weight, body fat loss and adult diseases positively.

• Journal of the East Asian Society of Dietary Life
• /
• v.17 no.4
• /
• pp.520-531
• /
• 2007

In this study, we found that the moisture content of black sesame seeds increased in proportion to steaming time, and it decreased in reverse proportion to the roasting temperature and time. The crude fat and crude ash contents were shown to be in the order of steamed black sesame>raw black sesame and roasted black sesame>raw black sesame, Crude protein decreased in reverse proportion to the steaming time, and more crude protein was found in the raw black sesame samples than in the roasted black sesame samples. The contents of sesamin, sesamolin, and total lignans were shown to be in the order of roasted black sesame>raw black sesame>steamed black sesame. In the steamed black sesame samples, sesamin showed its highest level with 20 minutes of treatment, and sesamolin showed its highest level with 15 minutes of treatment. In the roasted black sesame samples, sesamin, sesamolin, and total lignans showed their highest levels with 15 minutes of treatment at $100^{\circ}C$. The phenolic compound content increased in proportion to steaming time in the steamed samples, and reached a peak after 15 minutes of treatment at $100^{\circ}C$, and then decreased after 20 minutes. SOD-like activity reached a peak after 15 minutes of treatment, and in the roasted sample it reached a peak after 15 minutes of treatment, and then decreased after 20 minutes. SOD-like activity was comparatively lower than tocopherol and higher than sesamol. The intensity of electron donating ability, following 30 minute treatments, was shown to be highest in the steamed black sesame samples after 25 minutes of treatment, and next highest in the roasted black sesame samples after 15 minutes at $100^{\circ}C$. The electron donating ability was comparatively lower than tocopherol and sesamol. With regard to lecithin's antioxidant effects, the steamed samples showed a higher oxidation restriction rate in proportion to time, and the roasted samples showed the highest rate after 15 minutes of treatment, and then decreased after 20 minutes of treatment. for hydroxyl radical scavenging, similar scavenging activity to tocopherol, and comparatively higher scavenging activity than sesamol, was shown in all samples: all samples showed scavenging abilities of 90% or higher. In summary, this study applied three different treatment methods to black sesame to determine the optimum treatment conditions, and also examined the antioxidant effects and functional characteristics. The results showed that roasting can be used for other purposes than producing oil, and also suggested that methods other than roasting can be used in a variety of ways in cooking. Also, the different treatment methods can be applied in cooking in a variety of ways, to enhance functionality and preference.

• Animal Bioscience
• /
• v.35 no.8
• /
• pp.1223-1234
• /
• 2022

Objective: The objectives of this study were to evaluate the effects of daily feed intake during the laying period on embryonic myogenic differentiation 1 (MYOD1), myogenic factor 5 (MYF5), and myogenic factor 6 (MYF6) gene expression in genetically fat and lean lines of chickens. Methods: An experiment in a 2×2 factorial design was conducted with two dietary intake levels (100% and 75% of nutrition recommendation) and two broiler chicken lines (fat and lean). Two lines of hens (n = 384 for each line) at 23th week of age were randomly divided into 4 treatments with 12 replicates of 16 birds. The experiment started at 27th week of age (5% egg rate) and ended at 54th week of age. Hatched eggs from the medium laying period were collected. Real time polymerase chain reaction analysis was used to analyse the MYOD1, MYF5, and MYF6 mRNA levels of E7, E9, E11, E13, and E15 body tissues and E17, E19, and E21 chest and thigh muscle samples. Results: The results indicated that there were significant effects of line, dietary intake, and interactions between them on MYOD1, MYF5, and MYF6 gene mRNA expression levels in embryonic tissues. Low daily feed intake did not change the expression trend of MYOD1 mRNA in either line, but changed the peak values, especially in lean line. Low daily feed intake altered the trend in MYF5 mRNA expression level in both lines and apparently delayed its onset. There was no apparent effect of low daily feed intake on the trends of MYF6 mRNA expression levels in either line, but it significantly changed the values on many embryonic days. Conclusion: Maternal nutrient restriction affects myogenesis and is manifested in the expression of embryonic MYOD1, MYF5, and MYF6 genes. Long term selection for fat deposition in broiler chickens changes the pattern and intensity of myogenesis.

• The Journal of the Korea Contents Association
• /
• v.8 no.10
• /
• pp.301-308
• /
• 2008

The purpose of this study was to investigate the change of physical fitness, MDA, SOD after short-term weight reduction through exercise, food restriction and exhalation of sweat among Taekwondo players. The subjects ten male collage Taekwondo players with excellent practical skills, and more than seven years of experiences. They were asked to reduce about 5% of their body weight in seven days. Physical fitness levels were analyzed through grip strength, muscle endurance, flexibility, power, agility, balance, reaction time and MDA and SOD through blood test. The results of this study were as follows; First, there was no significant change in physical fitness due to short-term weight reduction in muscle strength, muscle endurance, flexibility, power, agility, balance and reaction time. Second, The change of MDA and SOD, resulting from short-term weight reduction did not show significant difference statistically. Therefore, the results of this study indicate that the Taekwondo players' short-term weight reduction of 5% of their body weight did not influence on their athletic performance and physiological capacity. Therefore athletes and coaches should endeavor to take exercise, dietary, and dehydration into consideration when conducting 5% of their body weight reduction in a week.

• Toxicological Research
• /
• v.13 no.1_2
• /
• pp.95-101
• /
• 1997

Ethyl carbamate (EC) is a potent teratogen in rodents and is present at low concentration in fermented foods and alcohol beverages. It has been well hypothesized that some metabolic products are responsible for the teratogenic effects of the compound. In the present study, the effects of phenobarbital (PB) on EC-induced embryotoxicity were investigated in SD rats. Six groups were constructed: EC 300 (EC 300 mg/kg/day), EC 600 (EC 600 mg/kg/day), EC 600+PB (EC 600 mg/kg/day and PB 80 mg/kg/day), PB (PB 80 mg/kg/day), DR (dietary restriction, 8 g/day/rat) and a control group. Rats of the EC 600+PB group were pretreated with phenobarbital intraperitoneally for three days to induce cytochrome P450 enzymes, followed by oral administration of EC for two consecutive days. The incidence of fetal deaths in the EC 600+PB group was higher than that of the EC 600 group(42.7 vs. 14.3%). The incidence of fetal realformations in the EC 600+PB group was higher than that of the EC 600 group (external; 7.0 vs. 4.1%, visceral; 31.4 vs. 11.3%, skeletal; 11.1 vs. 6.5%). There was no embryotoxicity in the control, EC 300, PB and DR groups. These results show that the pretreatment with phenobarbital augments EC-induced embryotoxicity in rats, indicating an evidence that metabolic activation by cytochrome P450 may be the major pathway of EC to its embryotoxic forms.