Kim, Sung Hoon;Lee, Sang Taek;Seong, Moon-Woo;Kim, Man Jin;Lee, Jun Hwa
Journal of The Korean Society of Inherited Metabolic disease
/
v.20
no.1
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pp.29-35
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2020
Joubert syndrome (JS) is a rare genetic disorder that is characterized by ataxia, hypotonia, developmental delay, respiratory abnormalities such as apnea-hyperpnea, and abnormal eye movements. The pathognomonic diagnostic finding is the "molar tooth sign" (MTS) on brain magnetic resonance imaging (MRI), described as cerebellar vermis hypoplasia or dysplasia, thick and horizontally oriented superior cerebellar peduncles, and an abnormally deep interpeduncular fossa. JS is characterized by genetic heterogeneity: pathogenic variants in over 30 genes have been identified to date. The CEP290 protein, which is on chromosome 12q21.3, is most frequently mutated in patients with JS, especially with renal involvement. Here, we report a case of JS in a 14-year-old male patient with end-stage renal disease. To the best of our knowledge, this is the first Korean report of a patient with JS due to CEP290 mutation (c.6012-12T> A) whose diagnosis was confirmed after repetitive MRI. We suggest consultation with an experienced neuro-radiologist and follow-up MRI studies to detect a "hidden" MTS if clinical findings suggest a diagnosis of JS. Furthermore, even in the absence of an MTS, whole exome sequencing should be considered.
The purpose of this study was to compare the anthropometry, nutrient intakes, dietary - related behaviors and health - related behaviors of male workers that were divided according to their alcohol consumption. The mean height, weight and BMI of all subjects were 172.0$\pm$5.8 cm, 67.8$\pm$9.3 kg and 22.9$\pm$2.6 kg/$m^2$, respectively. The WHR was higher in the heavy drinking group than in the other groups(p<0.01), while the other anthropometric indices were not significantly different among the three groups. The calorie intake was highest(p<0.001) in the heavy drinking group(3,270.4$\pm$686.5 kcal), followed by moderate(2,602.8$\pm$415.8 kcal) drinking group and light drinking group(2,341.5$\pm$449.4 kcal). As alcohol consumption increased, there was a decrease in the percent of energy derived from carbohydrates and lipids, while there was an increase in the percent of energy derived from proteins and alcohol(p<0.001). The heavy and moderate drinking groups had a highers intake of protein compared to the light drinking group, and the heavy drinking group had a higher intake of lipid compared to the light and moderate drinking groups(p<0.01). The intakes of vitamin $B_2$, niacin and phosphorus were lower in the moderate group than the other groups(p<0.01). The average MAR(Mean adequacy ratio) was 1.44$\pm$0.35 and the intake of all nutrients, except for folic acid, was much higher than the Korean RI(Recommended intake). The fruit intake frequency and sleeping hours were the lowest in the heavy drinking group(p<0.05) and the subjects preferred the following order of food products when they drink: meat(42.5%), fish & shellfish(30.0%), fruit & vegetable(22.5%) and others(5.0%) as a side dish. The average rates of smoking and exercise for all the subjects were 77.5% and 67.5%, respectively. The results of this study showed that heavy alcohol drinkers were more prone to abdominal obesity and related chronic degenerative diseases, indicating the need for extensive nutritional education for heavy alcohol drinkers.
Park, Seong-kuk;Jun, Moo-hyung;Lee, Hun-jun;Min, Won-gi;Yoon, Yong-dhuk
Korean Journal of Veterinary Research
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v.29
no.3
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pp.283-289
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1989
In order to measure in vitro cell mediated immunity in the guinea pigs sensitized with the killed bacilli of Mycobacterium bovis ($AN_5$), M avium (serotype 2), M tuberculosis and M intracellulare (serotype 8), leukocyte adherence inhibition (LAI) test was established using the antigens of purified protein derivatives (PPD) tuberculin. By using LAI test, specificity of cell-mediated immune responses of the guinea pigs inoculated with various Mycobacterium spp was investigated, and comparison between values of LAI and skin test was also made to evaluate the specificity of the newly designed test. The results obtained throughout the experiments were summarized as follows; 1. The optimal concentration of PPD antigens for LAI test was 1 to 2mg per ml of medium. 2. When the leukocytes of guinea pigs sensitized with both M bovis($AN_5$) and M avium (serotype 2) for 2 to 8 weeks were incubated with homologous or heterologous PPD antigens, mean values of LAI test were $61.2{\pm}11.2$ and $65.6{\pm}5.1%$ in homologous PPD antigens respectively, while $30.0{\pm}3.7$ and $32.8{\pm}5.7%$ in heteNlogous PPD antigens, showing the prominently high value of LAI in the homologous syst,em (p<0.01). 3. When the leukocytes of guinea pigs sensitized with both M tuberculosis and M intracellulare (serotype 8) for 2 to 8 weeks were incubated with homologous and heterologous PPD antigens, mean values of LAI test were $67.9{\pm}2.9$ and $66.9{\pm}5.0%$ in homologous PPD antigens, while $27.4{\pm}7.4$ and $24.4{\pm}7.1%$ in heterologous PPD antigens, showing the prominently high value of LAI in the homologous system (p<0.01). 4. Comparing with the specificity of LAI and skin tests on the basis of the value obtained from the homologous system, deviation of reaction was revealed to be 49.5 to 100.2 in LAI test, and -15.9 to 52.0 in skin test.
Lee, Deog-Yong;Kang, Sang-Gyun;Rayamajhi, Nabin;Kang, Milan;Yoo, Han Sang
Korean Journal of Veterinary Research
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v.48
no.4
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pp.441-449
/
2008
The genus Lactobacillus is the largest of the genera included in lactic acid bacteria and is associated with mucosal membranes of human and animal. Only a few Lactobacillus plasmid-encoded functions have been discovered and used. In this study, a novel plasmid (pILR091) was isolated from a wild L. reuteri isolated from pig and described the characteristics of its replicons, genetic organization, and relationship with other plasmids. After digestion of the plasmid, pILR091, with SalI, plasmid DNA was cloned into the pQE-30Xa vector and sequenced. The complete sequence was confirmed by the sequencing of PCR products and analyzed with the Genbank database. The isolate copy number and stability were determined by quantitative-PCR. The complete sequence of L. reuteri contained 7,185 nucleotides with 39% G-C content and one cut site by two enzymes, SalI and HindIII. The similar ori sequence of the pC194- rolling circle replication family (TTTATATTGAT) was located 63 bp upstream of the protein replication sequence, ORF 1. Total of five ORFs was identified and the coding sequence represented 4,966 nucleotides (70.4%). ORF1 of pILR091 had a low similarity with the sequence of pTE44. Other ORFs also showed low homology and E-values. The average G-C content of pILR091 was 39%, similar with that of genomic DNA. The copy number of pILR091 was determined at approximately 24 to 25 molecules per genomic DNA. These results suggested that pILR091 might be a good candidate to construct a new vector, which could be used for cloning and expression of foreign genes in lactobacilli.
Neutral salt-soluble, hot water-soluble and methanol-soluble materials (hereinafter referred to Fr. NaCl, Fr. HW and Fr. MeOH, respectively) were extracted from sclerotium of Grifola umbellata. The Fr. NaCl and Fr. HW did not show any direct cytotoxicity against NIH3T3, Sarcoma 180 and MCF-7, but Fr. MeOH showed cytotoxicity against these cell lines at the concentration of $1,000\;{\mu}g/ml$. Intraperitoneal injection with Fr. NaCl showed antitumor effect with life prolongation of 66.7% and decrease the number of Sarcoma 180 cells of 54.2% in mice inoculated with Sarcoma 180. Fr. NaCl improved the immunopotentiation activity through alternative complement pathway and the alkaline phosphatase activity by $85.05{\sim}88.73%$ and 6 folds, respectively. The number of peritoneal exudate cells and the circulating leucocytes were increased by 1.7 and 3.6 folds in the Fr. NaCl treating group compared with the control group, respectively. The weight of immunoorgans such as liver, spleen and thymus were also gradually increased. The hematological analysis of the Fr. NaCl group was similar with that of the control group. The total polysaccharide and protein contents of Fr. NaCl were 98.25% and 1.44%, respectively. These results indicate that the antitumor activity of Fr. NaCl was exerted through immunopotentiation, but not through cytotoxicity against the tumor cells.
CHO Deuk-Moon;KIM Doo-Sang;LEE Dong-Soo;KIM Hyeung-Rak;PYEUN Jae-Hyeung
Korean Journal of Fisheries and Aquatic Sciences
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v.28
no.1
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pp.49-59
/
1995
Nine species of edible seaweed [green laver (Monostroma nitidium) and sea staghorn ( Codium fragile) of green algae; sea mustard (Undaria pinnatifida), seaweed fusiforme(Hizikia fusiforme), gulf weed (Sargassum fulvellium), and sea tangle (Laminaria japonica) of brown algae; seaweed dilatata (Halimeniopsis dilatata), seaweed furcata (Gloiopeltis furcata), and laver (Porphyra tenera) of red algae] collected from Kijang, Chungmu, and Yosu in Korea, were examined and compared on their chemical and mineral composition depending on their harvesting season. Crude protein $(N\pm6.25)$ showed about $45\%$ on moisture free basis (the contents of every components described below are shown as moisture free basis) for laver and $30\%$ for green laver collected from ever seasons and sites. Sea tangle showed the highest content in crude lipid $(10\%)$ among brown seaweeds and green laver had $6\%$ of crude lipid regardless their habitats. Ash and carbohydrate in sea staghorn hold about $90\%$ of total solids but those levels were only $50\%$ in laver. The relationship between ash and carbohydrate content showed a tendency with reverse correlation. Mineral compositions were examined on green laver, sea mustard, and seaweed furcata collected from Kijang. Sodium was eminent element (1,798-7,334mg/l00g) followed by potassium. Magnesium and calcium content were low level compared with sodium and potassium. As a micro-element iron was appraised the highest status (165-330mg/100g) in green laver, however, iron in sea mustard and seaweed furcata was comparatively low amount (2.7-47.4mg/100g). The level of zinc was also comparatively high and that was varied on habitats. In conclusion, chemical compositions of these algae were distinctively varied on species and habitats, and mineral compositions were notably changed by the harvesting season.
The uptake of cadmium in animals is mainly accumulated in and affected to the liver and kidney by binding with red blood cells and serum albumin. The process accounts for more than 50% of the total accumulated cadmium in the body. The kidneys may be damaged without regarding the pathway uptake of cadmium. In a group of rats on supplements of 1% chlorella and 40 ppm cadmium, the concentration of cadmium in urine greatly decreased by 66% compared to control group, and the total synthesis of metallothionein decreased by 48.6% compared to control group. However, no previous study has assessed the protective effect on kidney damage induced by cadmium uptake through supplementation with chlorella. This study analyzed the biochemical marker for kidney damage in the rats after uptake of 40 ppm $CdCl_2$ and supplementation of the diet of Sprague Dawley (SD) rats with 1%, 5%, and 10% chlorella during 4 weeks. In a group of SD rats on supplementation with 1% chlorella and uptake of 40 ppm $CdCl_2,\;\beta_2$ microglobulin in the urine was found to be $3.1\pm0.6\;{\mu}g/L$, a decrease of 58% compared to a group of Sp rats on uptake of $CdCl_2$ only, in which the $\beta_2$ microglobulin was found to be $4.9\pm0.7\;{\mu}g/L$. According to the results of histopathological observation, the accumulation of mild and localized chronic inflammatory cells in kidney tissues was observed in 50% of the SD rats on uptake of cadmium only. In contrast, only 30% of the SD rats on supplementation with 1% chlorella and uptake of 40ppm $CdCl_2$, representing a histopathological abnormality, and there were no histopathological abnormalities at all in groups of SD rats on supplementation with 5% or 10% chlorella and uptake of 40 ppm $CdCl_2$. In conclusion, protein, calcium, and iron, which account for more than 50% of the total dried chlorella composition, may contribute to the reduction nephrotoxicity by stimulating both inhibited absorption of cadium and increased excretion of accumulated cadmium in kidneys.
Shin Jae Soon;Lee Seung Heon;Kim Won Ho;Yoon Sei Hyung;Kim Jong Geun;Nam Jin Woo
Journal of The Korean Society of Grassland and Forage Science
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v.25
no.2
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pp.113-118
/
2005
This experiment was conducted to compare the dry matter yields and fled values of winter forage crops using too cultivars of Barley, Rye md Italia ryegrass at the Dae-Ho reclaimed tideland, Korea from 2002 to 2004 2rowing season. Soil salt contents of three forage crops showed highest at sowing time, respectively and after wintering continuously lowed till harvest time. The dry matter yield was 6,668.8, 4,455.6 md 2,591.2 kg / ha, respectively, for Italian ryegrass, Barley and Rye. The highest Crude protein(CP) content was recorded in Italian ryegrass. Acid detergent fiber(ADF) contents were lowed in row with Barley, Italian ryegrass and Rye. Sodium contents in plant tissue were recorded high in line with Barley, Italian ryegrass and Rye. results mentions above suggest Italian ryegrass is suitable winter forage crops for cultivation on reclaimed tideland in view of the good emergence, forage production and its feed value.
This experiment evaluated the effects of dietary lysine restriction and energy density on growth performance, nutrient digestibility and meat quality of finishing pigs. A $2{\times}2$ factorial arrangement of treatments was utilized in a randomized complete block (RCB) design, and factor 1 was lysine restriction and factor 2 was energy density. The control diet was formulated to contain 3.265 Mcal of ME/kg, 0.75% lysine in the early-finishing phase and 3.265 Mcal of ME/kg, 0.60% lysine in the late-finishing phase and other nutrients met or exceeded NRC (1998) standards. Compared to the control diet (CON), lysine levels of experimental diets were restricted to 15% (treatment EL, EEL) or 30% (treatment ELL, EELL), whereas energy level of experimental diets was increased by 0.100 or 0.200 Mcal of ME/kg. A total of 100 crossbred pigs ([Yorkshire${\times}$Landrace]${\times}$Duroc), with average initial body weight of $58.47{\pm}1.42\;kg$, were allotted to 5 dietary treatments based on sex and body weight. Each treatment had 5 replicates with 4 pigs (two barrows and two gilts) per pen. ADG, ADFI and feed efficiency were calculated in an 8-week growth trial. In the late finishing period (5-8 weeks), pigs fed ELL or EELL diets had decreased ADG and feed efficiency (p<0.01), however, when the EEL diet was provided, a similar growth performance was observed compared to those fed the CON diet during the whole experimental period (p>0.05). In a metabolic trial, 15 pigs were used to evaluate the effect of dietary lysine restriction and energy density on nutrient digestibility. The digestibility of dry matter, crude fat and crude ash was not improved by restricting dietary lysine or energy density. However, crude protein digestibility was decreased (p<0.05) as dietary lysine was restricted. When dietary lysine was restricted, fecal nitrogen was increased whereas nitrogen retention was decreased. BUN concentration was affected by dietary lysine restriction; treatments ELL and EELL had higher BUN values than other treatments (p<0.01). Carcass characteristics and meat quality were measured when average body weight of pigs reached $107.83{\pm}1.50\;kg$. Treatment ELL had higher last rib backfat depth (p<0.05) than treatment CON, but ELL and EEL did not differ significantly. The ELL and EEL treatments had higher (p<0.05) subjective marbling score than treatment CON. Treatment EEL showed higher longissimus fat content than treatment EL and CON (p<0.01). The results indicated that finishing pigs fed a diet with 15% lysine restriction and 3.465 Mcal of ME/kg energy density had no detrimental effects on growth performance and N utilization, and could achieve substantial increases in marbling and longissimus fat content of pork.
Ahmadi, Farhad;Lee, Won Hee;Oh, Young-Kyoon;Park, Keunkyu;Kwak, Wan Sup
Asian-Australasian Journal of Animal Sciences
/
v.33
no.3
/
pp.446-455
/
2020
Objective: Our recent series of laboratory- and large-scale experiments confirmed that under aerobic and anaerobic conditions, sodium metabisulfite (SMB) was effective in preserving nutrients and antioxidant capacity of highly perishable fruit and vegetable discards (FVD). Hence, the purpose of this study was to examine how partial inclusion of SMB-treated FVD in total mixed ration (TMR) influences in vitro ruminal fermentation, whole-tract digestibility, nitrogen metabolism, blood metabolites, and voluntary feed intake of sheep. Methods: The FVD were mixed thoroughly with 6 g SMB/kg wet biomass and kept outdoors under aerobic conditions for 7 days. Four TMRs including four levels of SMB-treated FVD (as-fed basis) at 0%, 10%, 20%, and 30% (equaling to 0%, 1.9%, 3.8%, and 5.7% on dry matter basis, respectively), were prepared as replacement for corn grain. The ruminal fermentation metabolites were studied using an in vitro gas production test. Four mature male Corriedale sheep were assigned at random to the 4 diets for two separate sub-experiments; i) digestibility trial with four 21-d periods, and ii) voluntary feed intake trial with four 28-d periods. Results: Inclusion of SMB-treated FVD in the TMR tended to quadratically increase partitioning factor. No effect was seen on total-tract digestibility of organic matter, ether extract, crude protein, and acid detergent fiber, except for neutral detergent fiber digestibility that tended to linearly increase with increasing SMB-treated FVD in the TMR. The progressive increase of FVD preserved with SMB in the diet had no effect on nitrogen metabolism. Treatment had no effect on serum antioxidant capacity and blood metabolites assayed. Voluntary feed intake was not impaired by inclusion of SMB-treated FVD in the TMR. Conclusion: It appears that FVD preserved with SMB can be safely incorporated into TMR as replacement of corn grain without impairment of nutrient metabolism and feed intake.
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