• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.721-724
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• 2009

Meningococcal infections can be associated with abnormalities of the complement system, which contains 5 terminal complement proteins. Furthermore, deficiencies in 1 of these 5, complement component 7 (C7), leads to the loss of complement lytic function, and affected patients show increased susceptibility to recurrent meningococcal meningitis and systemic Neisseria gonorrhoeae infection. In September 2003, an 11-year-old female patient presented at our outpatient department with high fever, lower leg pain, headache, and petechiaes. She rapidly progressed to coma but later achieved full recovery due to prompt treatment. Her final diagnosis was meningococcal sepsis and arthritis. Her elder brother also had a similar bacterial meningoencephalitis history, which encouraged us to perform analyses for complement component and gene mutations. Resultantly, both the brother and sister were found to have the same mutation in the C7 gene. Subsequently, vaccinations of the meningococcal vaccine meningococcal vaccine ($Menomune^{(R)}$) were administered. However, in September 2006, the brother expired due to acute micrococcus meningoencephalitis. At present, the 16-year-old female patient is healthy. Here, we report a Korean family with a hereditary C7 deficiency with susceptibility to meningococcal infections due to C7 gene mutation.

• Journal of Life Science
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• v.14 no.1
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• pp.26-31
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• 2004

Redox factor-1 (Ref-1), known as a redox regulator, controls the DNA binding of AP-1 and is activated in HT29 colon cancer cells by hypoxia in vitro. REF-1 also increases tile DNA binding affinity of Hypoxia-inducible Factor-lalpha$ (HIF-lalpha$), HIF-like Factor (HLF) and early growth response-1 (Egr-1) which induce expression of the genes involved in angiogenesis, so that we speculate that REF-1 may play a role in hypoxia-induced angiogenesis. In this research we tried to detect novel proteins interacting with REF-1 using Yeast two-hybrid system using full-length REF-1 cDNA as bait. As result of such screening we detected 3 positive clones. DNA sequencing and GeneBank search revealed that one of the clones contained the same sequences as M.musculus cDNA for tioredoxin.

• Journal of Life Science
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• v.30 no.3
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• pp.285-290
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• 2020

Foot-and-mouth disease virus (FMDV), a member of the genus Aphthovirus in the Picornaviridae family, affects wild and domesticated ruminants and pigs. FMDV causes various clinical symptoms, including severe inflammation in infected tissue. Genome RNA of FMDV shows a positive single-strand chain approximately 8.3 kb long and encodes a single long open reading frame (ORF). The ORF is translated into structural and non-structural proteins by viral proteases. The FMDV 2C protein is one of the non-structural proteins encoded by FMDV and plays a critical role in FMD pathogenesis, including inflammation, apoptosis, and viral replication. In this study, we examined whether FMDV 2C induces intracellular expression of pro-inflammatory cytokine tumor necrosis factor alpha (TNFα). FMDV 2C expression in pig IBRS-2 cells increased mRNA and protein expression of TNFα at the transcriptional level via activation of TNFα promoter. Treatment with 4-phenylbutyric acid, an endoplasmic reticulum (ER) stress reducer, decreased TNFα expression induced by FMDV 2C. Activating transcription factor 4 (ATF4), a transcription factor mediating ER stress response, induced transactivation of TNFα promoter and expression of mRNA and protein of TNFα. However, the dominant negative mutant of ATF4 did not induce FMDV 2C-mediated TNFα expression. The results indicate that FMDV 2C protein increases clinical inflammation via ATF4-mediated TNFα expression and is associated with ER stress induction.

• Journal of Nutrition and Health
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• v.48 no.4
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• pp.319-326
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• 2015

Purpose: Borage oil (BO) and safflower oil (SO) are efficacious in reversing epidermal hyperproliferation, which is caused by the disruption of epidermal barrier. In this study, we compared the antiproliferative effect of dietary BO and SO. Altered metabolism of ceramide (Cer), the major lipid of epidermal barrier, was further determined by measurement of epidermal levels of individual Cer, glucosylceramide (GlcCer), and sphingomyelin (SM) species, and protein expression of Cer metabolizing enzymes. Methods: Epidermal hyperproliferation was induced in guinea pigs by a hydrogenated coconut diet (HCO) for 8 weeks. Subsequently, animals were fed diets of either BO (group HCO + BO) or SO (group HCO + SO) for 2 weeks. As controls, animals were fed BO (group BO) or HCO (group HCO) diets for 10 weeks. Results: Epidermal hyperproliferation was reversed in groups HCO + BO (67.6% of group HCO) and HCO + SO (84.5% of group HCO). Epidermal levels of Cer1/2, GlcCer-A/B, and ${\beta}$-glucocerebrosidase (GCase), an enzyme of GlcCer hydrolysis for Cer generation, were higher in group HCO + BO than in group HCO, and increased to levels similar to those of group BO. In addition, epidermal levels of SM1, serine palmitoyltransferase (SPT), and acidic sphingomyelinase (aSMase), enzymes of de novo Cer synthesis and SM hydrolysis for Cer generation, but not of Cer3-7, were higher in group HCO + BO than in group HCO. Despite an increase of SPT and aSMase in group HCO + SO to levels higher than in group HCO, epidermal levels of Cer1-7, GlcCer-A/B, and GCase were similar in these two groups. Notably, acidic ceramidase, an enzyme of Cer degradation, was highly expressed in group HCO + SO. Epidermal levels of GlcCer-C/D and SM-2/3 did not differ among groups. Conclusion: Dietary BO was more prominent for reversing epidermal hyperproliferation by enhancing Cer metabolism with increased levels of Cer1/2, GlcCer-A/B, and SM1 species, and of GCase proteins.

• Journal of Life Science
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• v.23 no.3
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• pp.347-354
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• 2013

Innate immunity is the ability to differentiate infectious agents from self. The innate immune system is comprised of a complicated network of recognition and effector molecules that act together to protect the host in the early stage of an infectious challenge. Mannose-binding lectin (MBL or mannose-binding protein, MBP) belongs to the family of $Ca^{2+}$-dependent lectins (C-type lectin with a collagen-like domain), which are considered an important component of innate immunity. While it is associated with increased risk and severity of infections and autoimmunity, the most frequent immuno-deficiency syndrome was reported to be low MBL level in blood. Deficiency of human MBL is caused by mutations in the coding region of the MBL gene. Rat homologue gene of human MBL gene was used to study functions of wild type and mutant MBL proteins. Although extensive studies have yielded the structural information of MBL, the functions of MBL, especially mutant MBL, still require investigation. We previously reported the cloning of rat wild-type MBL gene and the production of a truncated form of MBL protein and its antibody. Here, we present the cloning of mutant MBL cDNA in collagen-like domain (R40C, G42D, and G45E) using site-directed mutagenesis and differential behaviors of wild type and mutant MBL in cells. The major difference between wild type and mutant MBL was that while wild type MBL was secreted, mutant MBL was inhibited for secretion, retained in endoplasmic reticulum, and still functioned as a lectin.

• The Journal of the Korean dental association
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• v.10 no.5
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• pp.307-316
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• 1972

신생 Ball/c Strain 종 웅성백서 28두를 사용하고, 28두중 14두는 실험군에, 나머지 14두는 대조군에 각각 배당하였다. 실험군과 대조군은 동물희생시간(30분, 1시간, 4시간, 8시간, 12시간, 24시간 48시간)에 따라서 7군으로 구분하고, 각군에 2두식 배정하였다. 실험군은 백서를 Bell Jar내에 넣고, 여기에 창소를 서서히 넣어 Westinghouse Oxygen Analyzer로 30내지 40ppm이 되도록 하고 15분 동안 방치한 후, 이를 밖으로 제거하여 신선한 공기를 5분동안 마시게 하였다. 이런 과정을 반복하여 결국 55분 동안에 45분간 무 산소 상태에 있게 하였다. 악하선의 소편을 2% Paraformaldehyde(0.1M Cacodylate fuffer) 용액에 4시간동안 고정하였고, 탈수후 전현 표본포리법에 따라 Epoxy유지를 혼합하여 이몰하였다. LKB Ultratome으로 1μ의 연속절편을 만들었고 야기시킨후 신생백서의 악하선세포에 세포학적변화를 보고저하였고, 기결과를 요약하면 다음과 같다. 1. 신생 백서에 산소결핍을 야기시켰을때에 나타나는 초기의 세포학적변화는 이를 이르킨 후 1시간과 4시간사이가 절정에 달하였다. 이실험에서 유해적 영향은 공포화하고 점액성 및 장액성세포의 핵이 농축변성하며 장액성분필과기의 방출이 지연되어 세포질첨단에 축적되었다. 이들의 회복은 적어도 4시후에 시작되과, 12시간에는 세포질의 대부분, mrgl 핵의 용태가 정상으로 되돌아간다. 24시간에는 실험군과 대조군간에 있어서 야간의 차이는 인정되나 선소엽은 아직 초기에 볼수있었든 퇴행성변화의 잔여물을 함유하고 있었다. 2. 위에 요약한 본연구의 결과는 노인들이 보고한 산소결핍증이 악하선에 있어서도 단백질합성의 억제현상을 나타냈음을 세포학적으로 확증한 것이라하겠다.

• Journal of the Korean Society of Food Science and Nutrition
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• v.30 no.4
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• pp.739-746
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• 2001

To assess the nutrient intakes and diet quality of middle school students living in Kangneung area a dietary survey using th 3-day food record was conducted with 226 subjects. Mean daily energy intake was 2114$\pm$502 kcal with 61.6% of energy supplied by carbohydrate 16.4% by protein and 22.0% by fat. As well as insufficiencies in calcium and iron intakes the bioavailability of calcium and iron consumed is considered to low because most of them came from plant origins. For calcium iron vitamin A and vitamin B$_2$the proportions of subjects with intake levels less than 75% of RDA were all over 46%. The mean adequacy ratio(MAR) an index of overall dietary quality was 0.70 for males and 0.73 for females. The indexes of nutritional quality (INQ) were over 1 for most nutrients except 4 nutrients; 0.48 of calcium 0.64 of iron 0.73 of vitamin A and 0.79 of vitamin B$_2$. Nutrient intake levels less than 75% of RDA may be appropriate as a criteria for insufficiency of nutrient intakes. Based on these results it is evident that some of middle school students in Kangneung area did not consume enough nutrients especially calcium iron and vitamin A.

• Korean Journal of Poultry Science
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• v.11 no.1
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• pp.1-12
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• 1984

Since a 13% dietary protein level is generally accepted as a standard in evaluating net protein utilization values of protein sources in chicks, limiting amino acids a 13% protein basal diet containing 15% isolated soy-protein as the only source of dietary protein, were identified. Of such amino acids as methionine, lysine, threonine and tryptophan added to the basal diet singly or as a combination, methionine appeared as the only limiting amino acid for optimum growth of the chicks. When the requirement of total sulfur-containing acids (TSAA) was estimated as the point at which the dose-response curve intersected a line representing the plateau for maximum performance, the TSAA requirements for maximum growth and feed intake were 4.73% and 3.73% of dietary protein, respectively. The values, expressed in terms of TSAA intake, required for maximum weight gain, feed intake and gain/feed ratio were 167.1, 136.8 and 159.1 mg/bird/day, respectively.

• Journal of Life Science
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• v.19 no.4
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• pp.520-525
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• 2009

Early onset of Batten disease (EBD), one of the most lethal neurodegenerative storage disorders of childhood, is caused by inactivating mutations in the Ceroid Lipofuscinosis, Neuronal (CLN1) gene. Neurogranin, a calmodulin-binding protein, is expressed in the brain and participates in the protein kinase C (PKC) signaling pathway. While oxidative stress is the suggested cause of neurodegeneration in EBD, its molecular mechanism(s) remains obscure. In this research, we examined the levels of neurogranin in the brain mRNA of wild-type (WT) mice and EBD knockout (KO) mice, as well as the proteins. We also performed neuronal cultures to measure the expression levels of neurgranin and phosphorylated-neurogranin with or without oxidative stress inducers and anti-oxidants. Results showed that neurogranin in both EBD KO mice brain mRNA and protein extracts decreased in an age dependent manner. However, high amounts of phosphorylated-neurogranin were detected in the 6-month brain. This pattern was also confirmed by cultured neurospheres samples. Moreover, neurospheres treated with $H_2O_2$, an oxidative stress inducer, showed increased phosphorylated-neurogranin patterns. Interestingly, this pattern returned to normal status when treated with N-acetyl-L-cystein, an anti-oxidant, after $H_2O_2$ treatment was performed. Our results suggest that the phosphorylation of neurogranin is affected by oxidative stress status in EBD, and appropriate anti-oxidant treatment will relieve hyper-phosphorylation of neurogranin.

• Korean Journal of Microbiology
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• v.23 no.3
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• pp.172-176
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• 1985

The present study was designed to investigate isoenzyme (ACPase, ALPase) pattern and its refulatory function between catabolically repressed and derepressed states in yeast, Saccharomyces uvarum. As the results, no other isoenzyme was detectable in acid phosphatase, but there were three isoenzyme types in aldaline phosphatase. Type "B" isoenzyme among alkaline phosphatases in catabolically repressed cell was derepressed, but in normally cultivated cell, type "C" isoenzyme was derepressed while type "B" activity was lowered. Type "B" isoenzyme could be postulated as repressible enzyme, type "A" as constityityve enzyme and type "C" as L-histidinol phosphatase, respectively, Also, it could be shown that type "B" ALPase, repressible enzyme, compensated for phosphate group supplier under catabolically repressed states. Protein profile in cytoplasmic soluble fraction of exponential phase cell was characterized by negative charged protein.