Journal of the korean academy of Pediatric Dentistry
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v.26
no.1
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pp.151-156
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1999
Self-injurious behavior is defined as deliberate harm to one's own body without suicidal intent. It usually occurs as head banging or hitting, skin cutting, or finger biting and includes ocular, genital and oral self-mutilation. Lesch-Nyhan syndrome is a rare X-linked recessively inherited disorder, caused by complete absence of hypoxanthin-guanine phosphoribosyl transferase(HPRT) activity. Clinical presentation is characterized by mental retardation, chorea, athetosis, hyperuricemia, uricosuria and self-mutilating behavior. In these patients, the most typical feature is loss of tissue from biting themselves, even though they are not insensitive to pain. The dental management of self-mutilation includes treatment with appliances such as soft mouthguard or lip bumper, extraction of all the teeth, and orthognathic surgery. We report a 25-month-old boy who was a known case of Lesch-Nyhan syndrome and presented with severe self-mutilation wound on his lower lip. Vital pulpectomy and coronal resection was done as a more conservative approach than extracting all primary anterior teeth. Due to maintaining the root portion of the teeth in the bone, it is expected that the normal growth of the alveolar bone will be achieved.
Proceedings of the Korean Society of Plant Pathology Conference
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2003.10a
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pp.81.2-82
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2003
Plants have evolved along with pathogens, and they have developed sophisticated defense systems against specific microorganisms to survive. G-protons are considered one of the upstream signaling components working as a key for the defense signal transduction pathway. For activation and inactivation of G-protein, GTP-biding proteins are involved. GTP -binding proteins are found in all organisms. Small GTP-binding proteins, having masses of 21 to 30kD, belong to a superfamily, often named the Ras supefamily because the founding members are encoded by human Ras genes initially discovered as cellular homologs of the viral ras oncogene. Members of this supefamily share several common structural features, including several guanine nucleotide binding domains and an effector binding domain. However, exhibiting a remarkable diversity in both structure and function. They are important molecular switches that cycle between the GDP-bound inactive form into the GTP-bound active form through GDP/GTP replacement. In addition, most GTP-binding proteins cycle between membrane-bound and cytosolic forms. such as the RAC family are cytosolic signal transduction proteins that often are involved in processing of extracellular stimuli. Plant RAC proteins are implicated in regulation of plant cell architecture secondary wall formation, meristem signaling, and defense against pathogens. But their molecular mechanisms and functions are not well known. We isolated a RacB homolog from rice to study its role of defense against pathogens. We introduced the constitutively active and the dominant negative forms of the GTP-hinging protein OsRacB into the wild type rice. The dominant negative foms are using two forms (full-sequence and specific RNA interference with RacB). Employing southern, and protein analysis, we examine to different things between the wild type and the transformed plant. And analyzing biolistic bombardment of onion epidermal cell with GFP-RacB fusion protein revealed association with the nucle.
The Journal of Korea Assosiation for Disability and Oral Health
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v.2
no.2
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pp.147-152
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2006
Lesch-Nyhan syndrome is rare X-linked genetic disorder involving absence of the enzyme hypoxanthine guanine phosphoribosyl transferase (HGPRT) related to purine metabolism. The deficiency of HGPRT activity leads to an excesscive uric acid production and consequent hyperuricemia. It occurs almost exclusively in males, and the incidence is estimate to be 1/100,000~380,000. Clinical presentation is characterized by developmental delay, mental retardation, choreoathetosis, spastic cerebral palsy, nephrolithiasis, obstructive nephropathy and acute gouty arthritis. A characteristic feature of Lesch-Nyhan syndrome is the appearance of intractable self-mutilation behavior. Self-mutilation behavior is complicated by secondary infection and tissue loss as well as pain. The dental management of self-mutilation includes presertive methods of using appliances such as lip bumper or soft mouthguard and radical methods such as extraction of all teeth or orthognathic surgery. A case of Lesch-Nyhan syndrome patient with self-mutilation and severe lower lip injury is presented. He was treated successfully with soft mouthguard.
The Lesch-Nyhan syndrome which is caused by the deficiency of hypoxanthine guanine phosphoribosyltransferase is an X-linked recessive disorder characterized by hyperuricemia, choreoathetosis, mental retardation and compulsive self-injurious behavior. Clinical management of the patients with the Lesch-Nyhan syndrome is frustrating and requires burdensome medical treatment since it cripples the patient and shortens the life span by progression of neurological symptoms, but there are no cures or measures for relieving relentless natural course of the disease yet. Therefore, prenatal diagnosis of the affected fetus is important in genetic counselling for the family at high risk. In this study, four different mutations in the HPRT gene of four probands have been identified in four unrelated families; K215X, Q109X, nt.631 ${\Delta}A$, and nt.289 ${\Delta}GT$. Two mutations among them altered restriction enzyme sites; SpeI for Q109X and MaeI for nt.289 ${\Delta}GT$. Based on their molecular defects, prenatal diagnoses of 3 the fetuses were successfully made between ninth and eleventh week of gestation by polymerase chain reaction (PCR), restriction digestion and DNA sequencing using cDNA obtained from chorionic villus samples (CVS). We predicted the outcome of all fetuses prenatally. Among the three fetuses two were male and one was female according to the identification made by PCR amplification of the sex determining region of the Y chromosome(SRY) gene. Each carried a wild type allele for the corresponding mutant allele. They were also tested postnatally for the mutations to be unaffected.
We immunized BALB/c mice with purified Cucumber green mottle mosaic virus isolate HY1 (CGMMV-HY1). Through the selection of positive clones that were grown on the HAT medium, four sensitive monoclonal clones (CG99-01, CG99-02, CG99-03, and CG99-04) were selected from 500 Hypoxanthine-guanine phosphoribosyltransferase positive hybridoma cells. Four sensitive clones of CGMMV-HYI were determined as IgM type of the subclass of mouse immunoglobulins Ig group. The titer of monoclonal antiserum against CGMMVHY1 was estimated 1:12,800 by the indirect ELISA. Although monoclonal antibodies (MAbs) from CG99-01 and from CG99-04 cross-reacted with Zucchini green mottle mosaic virus and Kyuri green mottle mosaic virus, MAb from the cell line CG99-03 was highly specific to CGMMV. No MAbs cross-reacted with Cucumber mosaic virus-Fny. Only CG99-04 reacted with Pepper mild mottle virus weakly and CG99-02 reacted with both CGMMV and KGMMV. CGMMV was detected from the rind of watermelon fruit by DAS-ELISA of CGMMV-HY1, but not from the flesh of watermelon. Average seed transmission rate of CGMMV in watermelon was $24\%$ from symptomatic watermelon collected from 5 regions of Gyeongnam province. CGMMV was detected by DAS-ELISA with specific MAb of CGMMVHY1 periodically from root stock, during the sequential process for nursery seedling in Haman. Necrotic spots on cotyledons of root stock seedling progressed to reveal the typical symptomatology on the primary leaves of scion upon grafting. Here, we have established MAb based ELISA system, which could accurately detect CGMMV from watermelon seeds, nursery seedlings, transplants and field samples from greenhouse or open out door field as well.
The VP2 gene of infectious bursal disease virus (IBDV) Chinju which was previously detected in Chinju, Korea was cloned and sequenced to establish the information for the development of genetically engineered vaccines and diagnostic reagents against IBDV. The nucleotide sequence of the entire Chinju VP2 gene consisted of 1,356 bases long encoding 452 amino acids in a single open reading frame (ORF). It consisted of 368 adenine (27.1%), 363 cytosine (26.8%), 339 guanine (25.0%) and 286 thymine (21.1%) residues. The predicted $M_r$ of the Chinju VP2 protein was 48 kDa, and the protein contained 13 phosphorylation sites by protein kinase C, casein kinase II or tyrosine kinase, whereas 3 asparagine-linked glycosylation sites were recognized. The nucleotide sequence of Chinju VP2 ORF had a very close phylogenetic relationship with 98-99% homology to that of the very virulent IBDVs (vvIBDVs) HK46, OKYM, D6948, UK661, UPM97/61 and BD3/99. Also, the Chinju VP2 protein revealed a very close phylogenetic relationship with 99-100% homology to that of these vvIBDVs. The Chinju VP2 protein had 100% amino acid identity in the variable region of residues 206-360 with that of the D6948, HK46, OKYM and UK661, as well as 100% identity in two hypervariable regions of residues 212-224 and 314-324 with those of the D6948, HK46, OKYM, UK661, UPM97/61 and BD3/99. The amino acid sequence of the chinju VP2 protein contained a serine-rich heptapeptide of SWSASGS as in these vvIBDVs.
A number of epidemiological studies has indicated lifestyles including dietary habits are closely related to the development of certain forms of cancer. These findings have led several investigators to identify the ways in which these factors mdulate the risk of cancer. Seaweeds are rich sources of non-digestible polysaccharides which possibly posses physiological functions. In vitro studies showed several components in seaweeds inhibit tumor cell growth and mutagenicity of known food mutagens. On the other hand non-digestible polysaccharides of different food sources negatively affect mineral nutrition by decreasing mineral absorption. The objectives of this study was to investigate the effect of major seaweed intake on azoxymethane(AOM) - induced DNA damage a known cancer initiation step and on apparent absorption of calcium and iron. To accomplish these objectives twenty five ICR mice were divided into five groups and fed one of the following diets for 10 days : control diet d, diet containing 10% water-soluble fraction of seamustard or seatangle diet containing 10% water-insoluble fraction of seamustard or seatangle. AOM was injected 6 hours before sacrifice and N7-methylated guanines from the colonic DNA were quantified using a gas chromatography -mass spectroscopy. Fecal samples were collected on days 4 and 8. Caclium and iron contents of the diets and feces were analyzed using an atomic absorption spectrophotometry to determine the apparent absorption of these minerals. Results are as follows. AOM-induced guanine methylation of colon was decreased in animals fed diets containing water-soluble fractions of seamustard or seatangle compared to those in animals fed control diet although only the seatnagle fed group showed statistically significant effect. Apparent calcium absorption was significantly reduced in animals fed diets containing water-insoluble fractions of seaweeds. Iron absorption was significantly decreased and negatively balanced in animals fed diets containing water-insoluble fractions of both seaweeds, and water-soluble fraction of seatangle. In conclusion, seamustard and seatangle intakes may effectively prevent colon tumorigenesis by reducing a carcinogen-induced DNA damages, and more mechanistic studies on possible role of seaweeds on carcinogenesis are required. Also, adverse effects of seaweed diets cintaming a large amount of polysaccharides on mineral nutrition should be carefully monitored.
Kinetic parameters of purine nucleoside phosphorylase (PNP) from Saccharomyces cerevisiae were measured. The Michaelis constants determined for substrates of the enzyme were $ 2.0 * 10^{-4}$ M for inosine, $2.0 *10^{-3}$ M for deoxyinosine, $ 2.0 * 10^{-5}$ M for guanosine and $2.0 10 ^{-5}$ M for deoxyguanosine. According to the ratio of relative $K_{cat}$Km, substrate specificity of each nucleoside was in the order of guanosine or deoxyguanosine, inosine and deoxyinosine. Cosubstrate, phosphate, revealed downward curvature in Lineweaver-Burk plot at high concentrations, indicating a negative cooperativity between subunits. The inhibition constants for purine analogs were measured to be $ 6 * 10^{-4}$ M for formycin B as the competitive inhibitor of inosine, $ 9 * 10^{-6}$ M for guanine as the competitive inhibitor of guanosine, $2 * 10^{-4}$ M for hypoxanthine as the non competitive inhibitor of guanosine and $4.5 * 10 ^{-4}$ M for 6-mercaptopurine as the non competitive inhibitor of guanosine. Alternative substrates, guanosine, deoxyguanosine and adenosine were found to act as competitive inhibitors with Ki values o $f^ 2.0 * 10 {-5}$ M, $2.6 * 10^{-5}$ M and $8.5 * 10 ^{-4}$ M, respectively, when inosine was the variable substrate. Guanosine and deoxyguanosine were also observed as competitive inhibitors with the Ki values of $1.8 * 10^{-5}$ M and $ 3.0 * 10^{-5}$ M, respectively, when deoxyinesine was the variable substrate. The results of alternative substrate sstudies suggested that a single enzyme acted on different nucleosides, inosine, deoxyinosine, adenosine, guanosine and deoxyguanosine.e.
The interaction of the carcinogenic mycotoxin, Aflatoxin $B_1$, with the electron-donating molecule, benzene, was studied spectrophotometrically. The formation of charge-transfer complex between Aflatoxin $B_1$ and benzene in the presence of zinc chloride was observed and the apparent equilibrium constant of this charge-transfer complex was found to be 0.198 (liter $mole^{-1}$). It is assumed that, as the result of this study, some charge-transfer complexes could be formed between the weak electron-accepting Aflatoxin $B_1$ and strong electron-donating molecules, and the spectral changes occurred in the binding of Aflatoxin $B_1$ with proteins or DNA is attributed to the existence of charge-transfer type interaction.
SHIM, Kil Bo;JEONG, Yeon Gyeom;LEE, Heon Suk;JANG, Mi Soon
Korean Journal of Fisheries and Aquatic Sciences
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v.53
no.3
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pp.417-422
/
2020
We developed a seasoning sauce using hot water extracts from anchovy Engraulis japonica fish sauce processing by-products. A temperature of 121℃ was maintained for 120 min and the resulting amino acid content, salinity, and pH were 183.6 mg/100 g, 6.86, and 17.4 g/100 g, respectively. Radish juice, sea tangle Saccharina japonica extract, and mushroom Lentinula edodes were added to improve the flavor. The glutamic acid content of the extract mixed with 10% sea tangle extract was 88.87 mg/100 g and the 5'-GMP (guanine 5'-monophosphate) content of the extract mixed with 10% mushroom extract was 9.67 mg/ g. This study was conducted to determine optimal processing conditions for seasoned products using response surface methodology (RSM). The optimal conditions for X1 (sea tangle extract concentration) and X2 (mushroom extract concentration) were 15.0% and 5.0%, respectively, and the predicted values of the multiple response optimal conditions were Y1 (5'-GMP: 17.36 mg/100 g) and Y2 (glutamic acid: 157.35 mg/100 g). Under the optimal conditions, the experimental values of Y1 and Y2 were 17.32 mg/g and 155.36 mg/100 g, respectively, which are similar to the predicted values. We confirmed the feasibility of developing a seasoning sauce using hot water extract from anchovy fish sauce processing by-products and additives.
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