• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.17 no.1
• /
• pp.31-37
• /
• 2017

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle that causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed-onset type. Neonatal CPS1D cases often present their symptoms within the first days of life. Delayed-onset cases are predominantly adolescents or adults, and infantile delayed-onset cases are rare. Severe hyperammonemia in the neonatal period leads to serious brain damage, coma, and death if not treated promptly. Therefore, early diagnosis and acute treatment are crucial. Despite the improvement of treatments, including continuous hemodialysis, ammonia-lowering agents, and a low-protein diet, the overall outcome of severe forms of hyperammonemia often remains disappointing. As the liver is the only organ in which ammonia is converted into urea, liver transplantation has been considered as an elegant and radical alternative therapy to classical dietary and medical therapies. However, liver transplantation has many disadvantages, such as a considerable risk for technical complications and perioperative metabolic derangement, especially in neonates. Additionally, there is a lack of suitable donor organs in most countries. According to recent studies, liver cell transplantation is a therapeutic option and serves as a bridge to liver transplantation. Here, we report a Korean CPS1D patient with novel mutations in CPS1 who was treated by liver cell transplantation after being diagnosed in the neonatal period and showed a good neurodevelopmental outcome at the last follow-up at six months of age.

• Journal of Dairy Science and Biotechnology
• /
• v.33 no.3
• /
• pp.179-196
• /
• 2015

Because of our aging population, there is increasing concern about the impact of dementia and age-related cognitive decline. Intense research efforts on effective dietary interventions for the prevention or amelioration of dementia and age-related cognitive decline have indicated that dairy products affect physiological health and potentially healthy brain function during aging. Milk is a rich source of proteins and peptides with nutritional and immunotropic activities. The preparation of biologically active proteins and peptides generally requires enzymatic degradation, chemical modification, or the addition of specific co-factors. Milk-derived preparations are widely available in the food industry in the form of hygiene products and infant formulas. However, milk-derived products could also be applied as preventive or therapeutic measures for a wide-range of pathological conditions not only in neonates and infants but also in adults, including the elderly. Because they have no adverse side effects, milk-derived proteins and peptides could be used as a supplementary treatment for dementia and age-related cognitive decline.

• Korean Journal of Poultry Science
• /
• v.36 no.1
• /
• pp.15-21
• /
• 2009

A 10-wk layer feeding trial was conducted to investigate the effects of high quality corn distiller's dried grains with solubles (DDGS) on performance, egg qualities, and yolk fatty acid composition. The economics of using DDGS in the Korean situation was also analyzed. A total of 216 Hy-line Brown layers, 23-wk of age, were employed in this trial consisting of three dietary treatments (0%, 10%, and 20% DDGS), and six replicates per treatment. All experimental diets were prepared as iso-protein (17%) and iso-calorie (2,780 kcal/kg). The use of DDGS up to 20% in layer diets did not affect the feed intake, laying rate, egg weight, and feed conversion ratio (P>0.05). At 5th and 10th wk of the trial, the eggshell color, albumen height, and Haugh unit were not influenced by the DDGS supplementation. At 5th wk of the trial, the eggshell qualities, like eggshell weight, eggshell thickness, and eggshell strength, were not affected by the DDGS feeding; however, these eggshell qualities were decreased at 10th wk due to the 20% DDGS feeding (P<0.05). Yolk color of DDGS 20% increased compared to DDGS 0% at 5th and 10th wk of the trial (P<0.05). At 10th wk, yolk/egg ratio of DDGS 20% decreased compared to DDGS 0% (P<0.05). The yolk fat content was not changed due to DDGS feeding. The monounsaturated fatty acid content of yolk decreased linearly by feeding DDGS (P<0.05). The yolk polyunsaturated fatty acid content of DDGS 20% increased significantly compared to DDGS 0%. The DDGS feeding was not found to affect the degree of yolk fat unsaturation. The cost of feed (￦/kg feed) decreased as the level of DDGS increased. The production costs of egg (￦/kg egg) were cheap in the order of DDGS 10%, DDGS 20%, and DDGS 0%, indicating that DDGS is a viable alternative feed ingredient to corn and soybean meal. In conclusion, high quality DDGS ($L^*$ 61.72) could be used economically up to 20% level without any harmful effect on laying performance: however, the use of DDGS up to 10% is more economical than DDGS 20%.

• Asian-Australasian Journal of Animal Sciences
• /
• v.23 no.4
• /
• pp.521-529
• /
• 2010

Two experiments were conducted to evaluate multi-microbe submerged liquid (SLF) and solid substrate (SSF) fermented probiotic products in broilers. The SLF and SSF probiotics were comprised of Lactobacillus acidophilus ($1.1{\times}10^9$ and $4{\times}10^8$ cfu/g), Bacillus subtilis ($1.1{\times}10^9$ and $4{\times}10^9$ cfu/g), Saccharomyces cerevisiae ($1.5{\times}10^7$ and $1.0{\times}10^4$ cfu/g) and Aspergillus oryzae ($2.6{\times}10^7$ and $4.3{\times}10^7$ cfu/g), respectively. In Exp. 1, 640 day-old Ross chicks were allotted to 4 treatments, each comprising 4 replicates (40 chicks/replicate). The basal diet was prepared without any antimicrobials (negative control, NC), and 20 mg/kg avilamycin (positive control, PC), 0.3% SLF and 0.3% SSF probiotics were added to the basal diets as treatments. Birds fed PC and SSF diets showed improved (p<0.001) overall weight gain and F/G than birds fed NC and SLF diets; whereas, birds fed SLF diet had better weight gain and F/G than birds fed NC diet. Retention of CP was higher (p<0.05) in birds fed the SSF diet than birds fed PC, SLF and NC diets. Birds fed the SLF diet tended to have higher (p<0.10) cecal total anaerobic bacteria than birds fed PC and NC diets; whereas, lesser cecal coliforms were noticed in birds fed PC, SLF and SSF diets than birds fed the NC diet. In Exp. 2, 640 day-old Ross chicks were randomly allotted to 4 treatments in a $2{\times}2$ factorial arrangement. Each treatment had 4 replicates (40 chicks/replicate). Two different multi-microbe probiotic products (0.3% SLF or SSF) each with two different antibiotics (10 mg/kg colistin, or 20 mg/kg avilamycin) were used as dietary treatments. Birds fed the SSF diet had greater weight gain (p<0.001), better F/G (p<0.05), greater retention of energy (p<0.001) and protein (p<0.05), and lesser cecal Clostridium (d 35) than birds fed SLF diet. Birds fed the colistin-supplemented diet had less (p<0.01) cecal coliforms when compared with birds fed the avilamycin diet. Additionally, birds fed the avilamycin diet had greater energy retention (p<0.05) than birds fed the colistin diet. Thus, the results of this study suggest the multi-microbe probiotic product prepared by a solid substrate fermentation method to be superior to the probiotic product prepared by submerged liquid fermentation; moreover, feeding of probiotics with different antibiotics did not elicit any interaction effect between probiotic and antibiotic.

• Journal of the Korean Society of Food Science and Nutrition
• /
• v.36 no.11
• /
• pp.1391-1398
• /
• 2007

We performed a randomized double-blind placebo-controlled trial to determine whether Laminaria japonica extract (LJE) supplement modulates blood glucose, serum lipids and antioxidant systems in type II diabetic patients. We also measured critical parameters assessing safety in liver and kidney functions after LJE supplement. A total of 37 patients (18 males and 19 females) were randomized to either LJE group or placebo group. The treatment group received four 350 mg of LJE capsules (1.4 g, total) per day for 12 weeks. The placebo group received the same dose of cellulose capsules. Baseline characteristics regarding general life style and dietary intake pattern were similar between the two groups. There were no significant influences of LJE supplement except for waist circumference on anthropometric parameters. As the whole, 12 weeks of LJE supplement resulted in a little decrease in fasting blood glucose (FBG) and glycated hemoglobin (HbA1c), but a significant decrease was not observed. Total cholesterol, LDL-cholesterol and triglyceride concentrations were significantly (p<0.05) lowered in LJE group. The antioxidant enzymes, glutathion peroxidase (GSH-px) and superoxide dismutase (SOD) levels were elevated in the LJE group (p<0.05) compared to the placebo. The increase of these enzymes was associated significantly with the decrease of MDA concentration (p<0.05). Furthermore, LJE supplement showed no adverse effects on the functions of liver and kidney. Findings from this study suggest that LJE supplement can help improve serum lipid status in type II diabetic subjects without adverse effects.

• Development and Reproduction
• /
• v.10 no.1
• /
• pp.55-61
• /
• 2006

There is growing concern that dietary soy intake is associated with protection of breast cancer. However, questions persist on the potential adverse effects of the main soy constituent genistein(GS) on female reproductive physiology. In this study, we examined whether prepubertal exposure to GS affected on the onset of puberty and the associated reproductive parameters such as hormone receptor expressions in female rats. GS(100mg/kg/day) was administrated daily from postnatal day 25(PND 25) to the day when the first vaginal opening(VO) was observed, and the animals were sacrificed on the day after VO occurred. Gross anatomy and tissue weight were compared to test the GS's effect on the cell proliferation. Furthermore, histological studies were performed to assess the structural alterations in tissues. Specific radioimmunoassay(RIA) were carried out to measure serum LH levels. To determine the transcriptional changes in progesterone receptors(PR), total RNAs were extracted from ovary and uterus and were applied to semi-quantitative reverse transcription polymerase chain reaction(RT-PCR). As a results, advanced VO was shown in the GS group(PND $31.2{\pm}0.6$) compared to the vehicle group (PND $35.3{\pm}0.7$). GS treatment significantly increased wet weight of ovaries and uteri compared to the vehicle group. Increased serum LH levels were also shown in the GS group. Graafian follicles and corpora lutea(CL) were observed only in the ovaries from GS treated animals. Similarly, hypertrophy of luminal and glandular uterine epithelium were found only in the GS group. Collectively, these effects were probably due to the estrogenic effects of GS. In the semi-quantitative RT-PCR studies, the transcriptional activities of PR in both ovary and uterus from GS-treated group were significantly higher than those from the vehicle group. The present studies demonstrated that acute exposure to GS, at levels comparable to the ranges of human exposure, during the critical period of prepubertal stage activates the reproductive system resulting precocious puberty in immature female rats.

• Korean Journal of Poultry Science
• /
• v.29 no.3
• /
• pp.225-231
• /
• 2002

Total 240 of ISA Brown layers were employed in this experiment to study the effects of single or mixed feeding of Lactobacillus and yeast on the performance and intestinal microflora of laying hens. They were randomly allocated to six dietary treatments; None(Control), Pichia farinosa(PF), Lactobacillus crispatus avihen1 (LCH), Lactobacillus vaginalis avihen1(LVH), LCH＋PF, and LVH＋PF. Viable microflora were added to meet 3${\times}$10$\^$6/ cfu PF and 10$\^$7/ cfu Lacrobacillus per g of feed. There were four replicates per treatment, and 10 birds per replicates. Laying performance was recorded for 10 weeks, followed by a metabolism trial during which nutrient utilization, pattern of intestinal microflora and fecal NH$\sub$3/ emission were examined. Egg production and daily egg mass of birds fed either single or mixed microorganisms were significantly higher than those of the control(P<0.05). Egg weight and feed intake were not statistically different among all treatments. However, feed conversion ratio tended to improve by the supplementation of microbes. Digestibility of crude protein, ether extract and crude ash tended to improve in Lactobacillus treatments, however, there were not statistically different. With regards to the number of intestinal microbes, number of anaerobes were increased in microbes feeding group. Eggshell quality of PF layers was significantly poorer than those of the other treatments. No consistent trend was found in Haugh Unit among all treatments. Fecal NH$\sub$3/ gas emission was significantly lower in LVH, LVH＋PF and LCH＋PF than the other treatments(P<0.05). From the result of this experiment, it could be concluded that single or mixed feeding of Lactobacillus and yeast improves the laying performance and decreases the fecal ammonia gas emission. No synergic effect was found when both microbes were mixed and fed to the layers.

• Journal of the Korean Society of Food Science and Nutrition
• /
• v.33 no.7
• /
• pp.1212-1217
• /
• 2004

The aim of this study was to investigate Quality characteristics of Pleurospermum kamtschaticum to increase the value of functional food resources. To examine Quality characteristics of Pleurospermum kamtschaticum, various factors such as color, texture, fiber, minerals, tannin, crude proteins, crude lipids and sensory quality, were determined using physicochemical methods. The contents of dietary fiber were 0.57％ and 0.54％ in each 100 g of leaf and stem parts (freeze drying base). In mineral content, potassium was the highest value in both of leaf and stem parts (freeze drying base). The contents of tannin were 108.1 $\mu$g/mL and 20.9 $\mu$g/mL in leaf and stem parts (fresh base) respectively. Blanching stems showed significant difference in hardness from freeze-drying leaves. The antioxidative activity in ethanol extracts of Pleurospermum kamtschaticum was investigated using peroxide value and free radical scavenging activity. Free radical scavenging activity in ethanol extracts of Pleurospermum kamtschaticum was 43.5％ in a 100 $\mu$g/mL level, and the antioxidative index was 1.09 in a 500 $\mu$g/mL level. For functional food using Pleurospermum kamtschaticum the contents of crude proteins and lipids were about 9％ and 26％, respectively. The results from sensory evaluation of each treatment older age showed a better acceptability.

• Journal of Applied Biological Chemistry
• /
• v.53 no.2
• /
• pp.65-70
• /
• 2010

Medium-chain fatty acids (MCFA) are composed of 8-12 carbon atoms, and are found in coconut, cuphea, and palm kernel oil. MCFA were introduced into clinical nutrition in the 1950s for dietary treatment of malabsorption syndromes because of their rapid absorption and solubility. Recently, MCFA have been applied to Gastrointestinal Permeation Enhancement Technology (GIPET), which is one of the most important parts in drug delivery system in therapeutics. Therefore, to accumulate the MCFA in seed oil of rapeseed, much effort has been conducted by classical or molecular breeding. Laurate can be successfully accumulated up to 60 mol% in the seed oil of rapeseed by the expression of bay thioesterase (Uc FatB1) alone or crossed with a line over-expressing the coconut lysophosphatidic acid acyltransferase (LPAAT) under the control of a napin seed-storage protein promoter. Also, caprylate and caprate were obtained 7 mol% and 29 mol%, respectively, from plants over-expressing of the medium-chain specific thioesterase (Ch FatB2) alone or together with the chain-length-specific condensing enzyme (Ch KASIV). Despite the success of some research in utilizing parallel classical and molecular breeding to produce MCFA, commercially available seed oils have for the most part, not been realized. Recent research in the field of developing MCFA-enriched transgenic plants has established that there is no single rate-limiting step in the production of the target fatty acids. The purpose of this article is to review some of the recent progress in understanding the mechanism and regulation of MCFA production in seed oil of rapeseed.

• Korean Journal of Poultry Science
• /
• v.31 no.4
• /
• pp.265-271
• /
• 2004

A feeding trial was conducted to investigate the influence of feeding Lactobacillus reuteri culture (LR) on productive performance, intestinal microflora and availability in laying hens. Four hundred and eighty, Isa-Brown layers, 49 weeks of age, were fed diets supplemented with LR at the level of 0 (control), 0.1, 0.2, and $0.4\%$ of the diets for eight weeks. Egg production and egg weight were measured daily. Feed intake was weighed every two weeks. Egg quality was measured three times at the start, mid-term, and end of the experiment. Intestinal microflora were examined for Lactobacillus spp., E. coli and Salmonella at the end of the experiment. Overall egg production was the highest in $0.2\%$ LR (P<0.05), but that of $0.1\%$ or $0.4\%$ LR treatments did not significantly differ from that of control. Egg weight was significantly higher in LR feeding group than the control (P<0.05). Daily egg mass was significantly higher in $0.2\%$ and $0.4\%$ LR treatments compared to the control and $0.1\%$ LR (P<0.05). The number of jumbo and extra large eggs were increased in LR supplemented groups, especially in $0.1\%$ LR. Feed intake of layers fed LR supplemented diets tended to be lower than the control. However, feed conversion ratio significantly improved in LR supplemented groups (P<0.05). Availability of dry matter and crude protein improved significantly in $0.4\%$ LR treatment (P<0.05). But, those of ether extract and crude ash were not significantly different among treatments. Eggshell breaking strength and eggshell thickness were not significantly influenced by LR supplementation, and Haugh unit and yolk index were also similar to the control. Total number of Lactobacillus spp. in ileum and cecum fed LR supplemented diets were significantly higher than those of the control (P<0.05). There were no significant differences in intestinal E. coli and Salmonella in all treatments. Therefore, it is concluded that dietary supplementation of Lactobacillus reuteri culture can improve the laying performance, feed efficiency and intestinal Lactobacillus.