• 대한유전성대사질환학회지
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• 제6권1호
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• pp.96-107
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• 2006

Citrin deficiency resulting from mutations of SLC25A13is associated with two major clinical phenotypes; neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and adult-onset type 2 citrullinemia (CTLN2). In Korea, 7 cases of citrin deficiency have been diagnosed based on biochemical and molecular findings. Four NICCD cases were identified by newborn screening using MS/MS or presenting symptoms like cholestatic jaundice. They are all males, presenting with conjugated hyperbilirubinemia, elevated liver enzymes, hypoalbuminemia, mild hyperammonemia, elevated citrullin, methionine and threonine. All of them have been spontaneously recovered from hepatic manifestation by the age of 6-8 months. Mutation analysis has been performed using their genomic & cDNAs obtained from skin fibroblasts. They turned out to be compound heterozygotes carrying each of 851del4, IVS11+1G>A, and IVS13+1G>A. Three CTLN2 patients were identified. Two adult male patients presented with a sudden loss of consciousness, seizure, vomiting, hyperammonemia and citrullinemia in their twenties. They carried an IVS13+1G>A, 851del4, and IVS11+1G>A mutant alleles. The other CTLN2 patient was 52 year old female patient, manifesting lethargy, altered consciousness, irritability and hyperammonemia. Similar clinical symptoms had recurred at the delivery of first and second babies in her past medical history. She was managed by hemodialysis and survived with neurological sequellae. Also, we screened the presence of 9 common mutations in 500 Korean newborns using dried blood spot of filter papers. Only a allele carried 854del4 mutation. In conclusion, the entire picture of citrin deficiency in Korea including incidence, genotype, clinical features and natural courses, is still vague at the present time.

• 미생물학회지
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• 제5권1호
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• pp.20-23
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• 1967

I studied about temperature and pH concentration having influence upon chlorophyll decrease on the Glucose culture of Chlorella variegata Beijerinck(211/10a). I cultured under $25^{\circ}C$(standard), $15^{\circ}C$ and $35^{\circ}C$ and compared with each other. The culture of $15^{\circ}C$ didn't have any large difference with the standard culture($25^{\circ}C$) but the culture of $35^{\circ}C$ had a large amount of chlorophyll decrease without carbohydrate accumulation, stimulation of cell division and nitrogen-deficiency. Chlorella variegata had optimum pH 6.5-7 and was a little weak in all phenomenon under pH 8 rather than under pH 6.5-7. Under pH 5 they had deep chlorophyll decrease without phephytin.

• BMB Reports
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• 제54권6호
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• pp.317-322
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• 2021

CCCTC-binding factor (CTCF), a zinc finger protein, is a transcription factor and regulator of chromatin structure. Forebrain excitatory neuron-specific CTCF deficiency contributes to inflammation via enhanced transcription of inflammation-related genes in the cortex and hippocampus. However, little is known about the long-term effect of CTCF deficiency on postnatal neurons, astrocytes, or microglia in the hippocampus of adult mice. To address this, we knocked out the Ctcf gene in forebrain glutamatergic neurons (Ctcf cKO) by crossing Ctcf-floxed mice with Camk2a-Cre mice and examined the hippocampi of 7.5-10-month-old male mice using immunofluorescence microscopy. We found obvious neuronal cell death and reactive gliosis in the hippocampal cornu ammonis (CA)1 in 7.5-10-month-old cKO mice. Prominent rod-shaped microglia that participate in immune surveillance were observed in the stratum pyramidale and radiatum layer, indicating a potential increase in inflammatory mediators released by hippocampal neurons. Although neuronal loss was not observed in CA3, and dentate gyrus (DG) CTCF depletion induced a significant increase in the number of microglia in the stratum oriens of CA3 and reactive microgliosis and astrogliosis in the molecular layer and hilus of the DG in 7.5-10-month-old cKO mice. These results suggest that long-term Ctcf deletion from forebrain excitatory neurons may contribute to reactive gliosis induced by neuronal damage and consequent neuronal loss in the hippocampal CA1, DG, and CA3 in sequence over 7 months of age.

• 동아시아식생활학회지
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• 제22권4호
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• pp.463-472
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• 2012

This study was performed to investigate the iron status and its related factors in female college students residing in Gyeongnam. The prevalence of iron deficiency among subjects ranged from 3.4% in mean corpuscular hemoglobin concentration (MCHC) to 43.7% in ferritin. Weight, lean body mass (LBM) and body mass index (BMI) were positively correlated with ferritin concentration, but negatively correlated with total iron binding capacity (TIBC). Among the nutrients, vitamin A and B2 were major predictors of elevated iron status. Meal regularity was positively correlated with Fe and ferritin concentration, and meal number with transferrin saturation (TS), meal quantity with red blood cell (RBC) and hematocrit (Hct). Consumption of fruit, meat, fish and poultry showed strong positive correlation with hematological indices. Therefore, increasing vitamin A, B2, C, and iron intakes as well as maintenance of a healthy weight may be helpful in preventing iron deficiency in female college students.

• Journal of Microbiology and Biotechnology
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• 제26권7호
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• pp.1290-1302
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• 2016

Microalgae are being researched for their potential as attractive biofuel feedstock, particularly for their lipid production. For maximizing biofuel production, it is necessary to explore the effects of environmental factors on algal lipid-producing potential. In this study, the effects of nitrogen (N) sources (NO₂-N, NO₃-N, urea-N, NH₄-N, and N-deficiency) and carbon-to-nitrogen ratios (C/N= 0, 1.0, 3.0, and 5.0) on algal lipid-producing potential of Chlorella sp. HQ were investigated. The results showed that for Chlorella growth and lipid accumulation potential, NO₂-N was the best amongst the nitrogen sources, and NO₃-N and urea-N also contributed to algal growth and lipid accumulation potential, but NH₄-N and N-deficiency instead caused inhibitory effects. Moreover, the results indicated that algal lipid-producing potential was related to C/N ratios. With NO₂-N treatment and carbon addition (C/N = 1.0, 3.0, and 5.0), total lipid yield was enhanced by 12.96-20.37%, but triacylglycerol (TAG) yields decreased by 25.52-94.31%. As for NO₃-N treatment, carbon addition led to a 17.82-57.43%/25.86-82.67% reduction of total lipid/TAG yields. When NH₄-N was used as the nitrogen source, total lipid/TAG yields were increased by 46.67-113.33%/28.99-74.76% with carbon addition. The total lipid/TAG yields of urea-N treatment varied with C/N ratios. Overall, the highest TAG yield (TAG yield: 38.75 ± 5.21 mg/l; TAG content: 44.16 ± 4.35%) was achieved under NO₂-N treatment without carbon addition (C/N = 0), the condition that had merit for biofuel production.

• Childhood Kidney Diseases
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• 제7권1호
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• pp.86-90
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• 2003

저자들은 출생시부터 지속된 육안적 오렌지색소변 결정체를 주소로 내원한 8개월된 남아에서 HPRT 유전자의 돌연변이에 의한 HPRT 부분결핍증 1례를 경험하였기에 이를 문헌고찰과 함께 보고한다.

• Journal of Nutrition and Health
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• 제31권1호
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• pp.96-101
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• 1998

The prevalence of iron deficiency in later infancy and the toddler years(25% to 40% at 1 year of age) has not decreased remarkably , except in Western countries. The purpose of this study was to 1) determine the relationship between current feeding practices and iron status, and 2) assess compliance to infant feeding instructions. Two groupsof infants were examined. The first group of 302 infants aged 6 to 24months was seen at a well baby clinic while the second group of 135 infants of the same age group was assessed by venipuncture. Cutoff values for laboratory tests were as follows ; hemoglobin<11g/dL, mean corpuscular volume (MCV) <72fl ; red cell distribution width(RDW)>15% ; serum ferritin level<10ng/ml ; and transferrin saturation (serum iron(TIBC)<10%. The diagnosis of iron deficiency anemia (IDA) was made when a low hemoglobin level was associated with either low ferritin orlow transferrin saturation . Of the 302 children brought to the well baby clinic , 12.3%(n=37) were found to have anemia (hemoglobin<11.0/dL). In terms of children grouped according to feeding practices, it was found that children with anemial comprised 32.0% (24/75) of the prolonged breast-fed group (Group A), significantly more than the 4.0%(7/176) of the artificial milk feeding group(Group B). and 3.9%(2/51) of the switched from breast milk to iron -fortified weaning foods group(Group C).Among the 107 children with IDA , iron deficiency in 105 children(98.1%) was suggested by their dietary histories ; exclusive or prolonged breast-feeding for more than 6 months without iron fortification in 98 infants ; cow's milk consumption> 500ml/day without iron fortification during infancy(n=12), or >800ml without iron-fortified foods after infancy(n=15) ; and the use of unfortified forumula or unbalanced diets, mainly limited to rice gruel. Despite the relatively high (79.6%) motivation on the part of the infants mothers and supervison by professional personnel, the poor results in the infants receiving iron fortified foods were due to poor compliance(85.75). Among the mothers of 98 IDA patients who were contacted by telephone , it was revealed that 29% did not give the oral iron preparation for more than 2 months. Furthermore, negligence or disregard by the parents occurred in 14% of the case , discontinuance of the oral iron preparation by the parents due to side effects occurred in 6%, and the children's refusal or poor oral intake and no further trial occurred in 6%. The dietary history of a large group of infants was highly predictive of their risk for anemia . Continued consumption of breast milk until the age of 1 year is not warranted unless iron-fortified foods are given concomitantly. Because there is a problem with compliance, more successful and safe strategies for preventing iron deficiency woold included dual coverage in the from of therapeutic iron supplementation as well use of iron-fortified foods for teddlers who are at risk of iron deficiency.

• 동아시아식생활학회지
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• 제9권3호
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• pp.302-314
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• 1999

This study was conducted to evaluate nutrient intake, indices of anemia, serum lipids, and their correlations in 40 Korean female college students aged 21 to 25 years. Fasting blood samples were collected in October. 1997 Nutrient intake was investigated by 24-hour recall method for three days. Anthropometric measurements, blood pressure, iron status and serum lipids of the subjects were determined. The mean values for age. height, weight, BMI and blood pressure of the subjects were 22.1 years, 160.6cm, 54.4kg and 109.1/66.9mmHg, respectively. According to the body composition analysis, total body water, % body fat, lean body mass and WHR were 26.7L. 29.5%, 36.5kg and 0.8, respectively. Average daily intake of energy was 1,858.4kcal. Protein, phosphorus, vitamin B$_1$, vitamin B$_2$, niacin and vitamin C intakes were higher than the Korean recommended dietary allowence(RDA), whereas calcium, iron, vitamin A intakes were tower than that. Hemoglobin(Hb), hematocrit(Hct), serum iron(SI) and total iron binding capacity(TIBC) were measured and transferrin saturation (TS%) was calculated from serum. The mean values for Hb, Hct, SI, TIBC and TS were 13.6$\mu\textrm{g}$/㎗, 41.6%,97.8$\mu\textrm{g}$/㎗, 319.6$\mu\textrm{g}$/㎗ and 31.9%, respectively. The Prevalence rates of iron deficiency assessed by Hb and Hct were found to be 10% and 2.5%, respectively. However, when assessed with TIBC, the prevalence rate of iron deficiency was increased to 32.5%. The mean values for serum triglyceride(TG), total cholesterol(TC), HDL-Chol, LDL-Chol concentrations and atherogenic index(AI) were 154.7mg/㎗, 80.0mg/㎗, 47.4mg/㎗, 91.3mg/㎗ and 2.6, respectively. Correlations among indices of anemia. there were positive correlations between Hb and Hct, between SI and TS : negative correlations between TIBC and TS or SI. Correlations among serum lipids, there were positive correlations between TC and TG or LDL-cholesterol. Energy intakes were correlated positively with TIBC(p＜0.05), and vitamin C intakes negatively correlated with Hb(P＜0.05). And vitamin A intakes were negatively correlated with TG(p＜0.01). TC(p＜0.05) and LDL-Chol(p＜0.01). Hb negatively correlated with TG(P＜0.05). Body weights(p＜0.05), lean body mass(p＜0.05) and total body water(p＜0.01) werenegatively correlated with Hb. BMI and WHR correlated with TG and TC(p＜0.01). These results indicated that subjects with higher energy intake and overweight had a tendency to have iron deficiency. Increases in BMI and WHR were related to increases in serum lipids levels and decreases in TIBC. The results also showed that serum lipids were decreased when vitamin A and iron intakes were sufficient.

• KSBB Journal
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• 제10권4호
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• pp.349-356
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• 1995

하수처리장의 활생오니를 분리원으로 하여 수십종의 PHA 생산균을 분리하였다. 일반적으로 hy­d droxyvalerate monomer unit의 전구물질로 알려져 있지않은 glucose로부터 비교적 많은 PHA를 생산 하는 균주를 공시균으로 선정하여 형태학척, 배양적, 생리학적 제 특성을 검토한 결과 Pseudomonas 속으 로 통정되었다. 균체 생육을 위한 최적 배양온도 벚 배양 pH는 각각 $37^{\circ}C$와 7.0이었으며, 최적 탄소원 으로셔 glucose 1 %, 최적 질소원으로서 $(NH_4)_2SO_4$ 0.2%, K1HPO. 0.3%, $KH_2PO_4$ 0.45% 였다. 최적 P PHA 생산조건을 조사하기 위하여 2단계 배양법을 이용하였다 PHA 생산은 배지성분중 $NH_4, O_4$, Mg가 결핍되 였을 때 향상되었고, 그중 $NH_4$, 의 결핍시 PHA 축적률과 HV monomer의 함량이 가장 높았다. C/N molar ratio 95.2에서 PHA 축적률이 가장 높았다. 공시균주 Pseudomonas sp. HJ는 alkane, alkanoic acid, alcohol을 탄소원으로 하여 P PHB/HV를 생산하였다. PHA의 생산량과 HV monomer의 함량은 이용된 기질에 따라 다양하였으며, 특히 hexadecane와 propiOnate를 탄소원으로 하였을 때 PHA중의 HV monomer의 함량이 49~74mol %로 매우 높았다. IH-NMR로셔 공시균으로 부터 분리정제된 PHA의 조성을 분석한 결과 PHB/ H HV copolymer임을 알 수 였였다

• Journal of Nutrition and Health
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• 제3권3호
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• pp.149-159
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• 1970

This study is to investigate the status of anemia, especially iron deficiency anemia among pre-school children in rural area in Korea. The survey was conducted in Sang-dae Ri, Yusong Myon, Daedok Gun, Chung Chong Nam-Do from July 30 th to August 12th, 1968. The measurements were done of height, weight, hematologist and biochemical levels on ninety-two pre-school children, 47 male, and 45 female, one to six years of age. Hemoglobin was determined by the method of cyanmethemoglobin and hematocrit by micro hematocrit centrifuge. The determination of serum iron, iron-binding capacity was done by the method of Ramsay using bathophenanthroline and the serum albumin was determined by Biuret Reaction. The results of this study are as follows: 1) 54.4 percent of the pre-school children weighed less than 90 percent of the Korean General Standard Weight level. 2) The average hemoglobin level was $11.0{\pm}1.57gm/100ml$, 38.0 percent of the children were anemic with less than 1.0gm/100ml. Of the anemic children 60 percent were below the Korean General Standard Weight level. 3) 27.5 percent of the pre-school children were found to have below 32 percent of a hematocrit values and 28.0 percent showed less than 33 percent in M.C.H.C. These results showed that the incidence of hypochromic anemia in these pre-school children was high. 4) 37.9 percent of these children had a serum iron level less than $50{\mu}g/100ml\;and\;31.0\;percent\;had\;a\;TIBC\;above\;400{\mu}g$ while 48.3 percent showed a transferrin saturation lower than 15 percent. On the basis of these findings, it is concluded than the cause of this anemia was iron deficiency. 5) In this group there was a little evidence of low total serum protein levels. However, 10.4 percent of the children had a deficient serum albumin level, below 2.80 gm/100ml while 51.7 percent had a low level, less than 3.50gm/100ml, and 34.5 percent of the children had a low level of TIBC, less than $350{\mu}g/100ml$, and considering these facts, it is suggested that some of the anemias have a multiple causes through protein deficiency and repeated chronic infection apart from iron deficiency.