Journal of The Korean Society of Inherited Metabolic disease
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v.15
no.1
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pp.1-8
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2015
Purpose: 3-methylcrotonyl CoA carboxylase deficiency (3MCCD) is leucine metabolic disorder caused by mutation in MCCC1 or MCCC2 gene. Clinical manifestations are variable, ranging from fatal neonatal onset to asymptomatic individuals. There is no retrospective study of Korean patients undergoing long-term treatment for 3MCCD. We reported this study to find out clinical symptoms and gene analysis of 3MCCD patients. Methods: This study was based on data of patients diagnosed with 3MCCD in Soonchunhyang university hospital between April 2009 and September 2013. We report clinical, enzymatic and mutation data of 3MCCD patients found by newborn screening. Results: In tandem mass spectrometry, 3-OH-isovalerylcarnitine (C5OH) of all patients increased. And all 7 patients were elevated 3-methylcrotonylglycine (3MCG) and 3-hydroxyisovaleric acid (3HIVA) in urine. MCCC mutation was identified in 2 patients and MCCC2 was mutated in 5 patients. We found mutation occurred in 8 different parts of nucleotide and such mutation caused 7 different types of changes in amino acid. All patients are on medication of L-carnitine and L-glycine. 4 patients are taking biotin. And 4 patients are eating leucine free formula. After starting treatment, there were no significant changes of urine 3MCG and 3HIVA levels. Conclusions: According to our data, MCCC2 gene mutation was more common than MCCC1 gene mutation. But the level of 3HIVA or 3MCG in urine has no correlation with phenotype. All patients has no symptoms and are shown normal development.
Germinated brown rice (GBR) has received considerable attention over the last decade as a means of enhancing the nutritional value and health-promoting functions of rice. The effect of germination on the physicochemical and textural properties of brown rice (BR) was investigated in different rice varieties (Samkwang, Misomi, Chindeul, and Hyeonpum). Cooking properties, such as water absorption, expanded volume, and soluble solids were significantly increased by germination in all rice varieties. Textural properties (hardness, toughness, adhesiveness, and stickiness) of cooked samples were determined using a texture analyzer. Hardness and toughness were decreased by germination, whereas stickiness and adhesiveness increased significantly. These results revealed that germination leads to improvements in the cooking and eating properties of BR. In Rapid Visco Analyzer (RVA), significant reductions were observed for peak viscosity, break down, set back, and final viscosity after germination. Although the amylose content of Misomi was slightly decreased by germination, that of other varieties increased significantly. Germination induced no noticeable change in the average chain length of amylopectin in Misomi, Chindeul and Hyeonpum, but led to a significant increase in Samkwang.
Laminin, a kind of multidomain glycoproteins, is mainly localized in the basement membranes of various tissues. It is known that laminin plays an important part in mammalian lung morphogenesis. The authors have undertaken this study to investigate the changes in the distribution of laminin, and to find out cells which synthesize laminin during the organogenesis and differentiation of the lung. The fetal and neoantal rats (Sprague-Dawley strain) were used as experimental animals. The immunohisto-chemical methods were employed for detection of laminin within the developing lung tissue and the immunegold cytochemical methods were performed for detection of cells which synthesize laminin according to each stage of development. The results are as follows; 1. During fetal life, strong immunoreactivity for laminin is maintained in the basement membranes of the blood vessels and the bronchioles, the extracellular matrix of the mesenchyme, and basal lamina of the alveolar septum in the fetal rat lung. 2. After birth, laminin immunoreactivity at the alveolar septum is gradually reduced. 3. During fetal life, laminin is mainly detected within the cytoplasm of the mesenchymal cells, the endothelial cells of blood vessels and the fibroblasts in fetal rat lung. 4. According to the differentiation of type I and type II pneumocyte after birth, laminin is detected within cytoplasm of the type I pneumocytes, type II pneumocytes and fibroblasts. It is consequently suggested that laminin is largely expressed in the developing lung and laminin may be also synthesized by the type II pneumonocytes at early newborn stages.
Pemetrexed has demonstrated clinical activity in non-small cell lung cancer (NSCLC) as well as other solid tumors. It transports into the cells via reduced folate carrier (RFC) and is polyglutamated by folypolyglutamate synthetase (FPGS). Pemetrexed directly inhibits several folate-dependent enzymes such as thymidylate synthase (TS), dihydrofolate reductase (DHFR), and glycinamide ribonucleotide formyltransferase (GARFT). We investigated the effects of genetic variations and the expression of RFC, FPGS, TS and DHFR enzymes on drug sensitivity to pemetrexed in NSCLC cells. Polymorphisms in RFC, FPGS, and DHFR were genotyped in four NSCLC cells - A549, PC14, HCC-1588, and H226. Real-time RT-PCR and Western blot was performed to evaluate mRNA transcripts and protein of these genes. The cytotoxicity of pemetrexed was measured by SRB assay. In PC14 and H226 cells, increased mRNA expressions of RFC and FPGS were associated with higher cytotoxicity to pemetrexed. 2R/2R genotype of TS and its increased mRNA expression were associated with drug resistance to pemetrexed in A549 cells, whereas 3R/3R genotype in TS with decreased mRNA expression was associated with higher sensitivity in H226 cells. After pemetrexed treatment, an inverse change of DHFR mRNA and protein expression was found. The strongest linkage disequilibrium (LD) was discovered between-1726C>T and -1188A>C SNP of DHFR gene. Our findings suggest the cytotoxic effect of pemetrexed may be associated with genetic polymorphisms and the expression level of genes involved in pemetrexed metabolisms in NSCLC cells.
LEE Kang-Ho;CHO Ho-Sung;LEE Dong-Ho;KIM Min-Gi;CHO Young-Je;SUH Jae-Soo;KIM Dong-Soo
Korean Journal of Fisheries and Aquatic Sciences
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v.26
no.4
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pp.330-339
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1993
To optimize the processing conditions of fermented ascidian, Halocynthia roretzi, fermentation at low temperature with different salt contents, the effect of enzymes added, and the quality changes during fermentation were investigated. As the quality factors, changes in such components as free amino acid, volatile basic nitrogen(VBN), amino nitrogen, total creatinine, total carotenoid, extents of browning, reducing sugar and glycogen were determined. The quality was also evaluated organolatically by pannel test. Fresh deshelled and sliced ascidian were fermented for 50 days at $5{\pm}1^{\circ}C$ with different salt contents of 5, 10, $15\%$ (w/w) with enzyme contents of papain $0.1\%$ and protease-A $0.1\%$ VBN increased gradually during the 50 days of fermentation and showed $30{\sim}40mg/100g$ at 30, 35 and 45 days in case of salt contents 5, 10 and $15\%$ added with $0.1\%$ papain and protease-A, respectively. Amino nitrogen and the total creatinine increased until 20 days, hereafter tended to decrease gradually. Total carotenoid and glycogen also decreased during the fermentation. The results of sensory evaluation of fermented ascidian at $5{\pm}1^{\circ}C$ added $0.1\%$ papain or protease-A showed that the peculiar taste and flavor of ascidian was sustained for $30{\sim}40$ at least 20 days with $5\%$ NaCl and $35{\sim}45$ days of fermentation with 10 and $15\%$ NaCl.
Lactacystin, a microbial natural product synthesized by Streptomyces, has been commonly used as a selective proteasome inhibitor in many studies. Proteasome inhibitors is known to be preventing the proliferation of cancer cells in vivo as well as in vitro. Furthermore, proteasome inhibitors, as single or combined with other anticancer agents, are suggested as a new class of potential anticancer agents. This study was undertaken to examine in vitro effects of cytotoxicity and growth inhibition, and the molecular mechanism underlying induction of apoptosis in SCC25 human tongue sqaumous cell carcinoma cell line treated with lactacystin. The viability of SCC25 cells, human normal keratinocytes (HaCaT cells) and human gingiva fibroblasts (HGF-1 cells), and the growth inhibition of SCC25 cells were assessed by MTT assay and clonogenic assay respectively. The hoechst staining, hemacolor staining and TUNEL staining were conducted to observe SCC25 cells undergoing apoptosis. SCC25 cells were treated with lactacystin, and Western blotting, immunocytochemistry, confocal microscopy, FAScan flow cytometry, MMP activity, and proteasome activity were performed. Lactacystin treatment of SCC25 cells resulted in a time- and does-dependent decrease of cell viability and a does-dependent inhibition of cell growth, and induced apoptotic cell death. Interestingly, lactacytin remarkably revealed cytotoxicity in SCC25 cells but not normal cells. And tested SCC25 cells showed several lines of apoptotic manifestation such as nuclear condensation, DNA fragmentation, the reduction of MMP and proteasome activity, the decrease of DNA contents, the release of cytochrome c into cytosol, the translocation of AIF and DFF40 (CAD) onto nuclei, the up-regulation of Bax, and the activation of caspase-7, caspase-3, PARP, lamin A/C and DFF45 (ICAD). Flow cytometric analysis revealed that lactacystin resulted in G1 arrest in cell cycle progression which was associated with up-regulation in the protein expression of CDK inhibitors, $p21^{WAF1/CIP1}$ and $p27^{KIP1}$. We presented data indicating that lactacystin induces G1 cell cycle arrest and apoptois via proteasome, mitochondria and caspase pathway in SCC25 cells. Therefore our data provide the possibility that lactacystin could be as a novel therapeutic strategy for human tongue squamous cell carcinoma.
Journal of the korean academy of Pediatric Dentistry
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v.39
no.1
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pp.90-96
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2012
Supernumerary tooth occurs most frequently at premaxilla area. Followed by mandibular premolar area, mandibular fourth molar area, maxillary paramolar area. Mesiodens are mainly impacted in the palatal area and surgical approach is made at palatal side. The time of surgery remains controversial. In case of inverted or horizontal impacted supernumerary tooth, intraosseous tooth movement and vertical growth of premaxilla makes surgical extraction more difficult. And also the more quantity of removed bone is, the higher degree of difficulty is. Inverted mesiodens of these cases were impacted superior to apex level of adjacent permanent incisor. Although CT examination revealed exact location of impacted tooth, surgical procedure including ostectomy may take a long time more than expected. So, before surgical extraction, it's need to be considered several factors such as necessity of CT taking, degree of difficulty, direction of surgical approach, necessity of general anesthesia etc.
Background : The information on nasal transport and the metabolism of peptides have been obtained from pharmacokinetic investigations in experimental animals. However, there are no transport and metabolic studies of human nasal epithelial cells. In this study, the permeability characteristics and the metabolic properties of in vitro human nasal cell monolayers were investigated. Material and Methods : Normal human inferior nasal conchal tissue samples were obtained from patients undergoing endoscopic nasal cavitary surgery. The specimens were cultured in a transwell using an air-liquid Interface (ALI) culture, and the transepithelial electrical resistance (TEER) value of the blank filter and confluent cell monolayers were measured. To determine the % leakage of mannitol, $4{\mu}mol%$$^{14}C$-labelled mannitol was added and the % leakage was measured every 10 minute for 1 hour. Result : Human nasal epithelial cells in the primary culture grew to a confluent monolayer within 7 days and expressed microvilli. The tight junction between the cells was confirmed by transmission electron microscopy. The TEER value of the blank filter, fifth day and seventh day reached $108.5\;ohm.cm^2$, $141\;ohm.cm^2$ and $177.5\;ohm.cm^2$, respectively. Transcellular % leakage of the $^{14}$-mannitol at 10, 20, 30, 40, 50 and 60 minutes was $35.67{\pm}5.43$, $34.42{\pm}5.60$, $32.75{\pm}5.71$, $31.76{\pm}4.22$, $30.96{\pm}3.49$ and $29.60{\pm}3.68\;%$, respectively. Conclusion : The human nasal epithelial monolayer using ALI culture techniques is suitable for a transcellular permeability study. The data suggests that human nasal epithelial cells In an ALI culture technique shows some promise for a nasal transport and metabolism study.
There has recently been increasing interest in the use of NMDA receptor antagonists as potential neuroprotective agents for the treatment of ischemic stroke. To evaluate the neuroprotective effect of the selective non-competitive NMDA receptor antagonist MK-801 in focal cerebral ischemia, local cerebral glucose utilization (ICGU) was examined in 15 neuroanatomically discrete regions of the conscious rat brain using the 2-deoxy-D[$^{14}C$] glucose quantitative autoradiographic technique 24 hr after left middle cerebral artery occlusion (MCAO). Animals received MK-801 (5 mg/kg i.v.) or saline vehicle before (20-30 min) or after (30 min) MCAO. Both pretreatment and posttreatment of MK-801 increased occluded/non-occluded ICGU ratio in 7 and 5 of the 15 regions measured, respectively (most notably in cortical structures). Following MK-801 pretreatment, there was evidence of widespread increases in ICGU not only in the non-occluded hemisphere (12 of the 15 areas studied) but also in the occluded hemisphere (13 of the 15 areas studied), while MK-801 postreatment did not significantly increase ICGU both in the normal and occluded hemispheres. These data indicate that MK-801 has a neuroprotective effect in focal cerebral ischemia and demonstrate that MK-801 provides widespread alterations of glucose utilization in conscious animals.
Journal of the Korean Institute of Traditional Landscape Architecture
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v.38
no.1
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pp.45-52
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2020
This study analyzed the components contained in the pine needles of first and second-year-olds to analyze the factors that the in vivo content of inorganic elements affects the sex determination of pine trees. In response, the plan for pine tree plant and maintenance was intended to be presented in consideration of the reproductive environment and physiological characteristics. The results are as follows. First, last year, when there were many encyclopedias, the analyzed N(%) content was found to be high. The nitrogen content of the previous year's soil was found to affect the production of the spheres the following year. This is believed to be possible to reduce the rate of Pine pollen produced in the new plant in the following year through a dispute over quality consumption in the spring of the previous year. Second, the weapons elements involved in the Pine cones and the generation of the Pine pollen in the new plant appeared to be P(%), K(%), Ca(%), and Fe(%). However, the nutrients from the previous year's leaves of Ca(%) and Fe(%) were found to have a low influence on the sex determination of first-year pine trees. Because Ca(%) and Fe(%) are not able to move nutrients accumulated in aging organs due to the nature of the components, feeding nutrients in the fall when the growth of the previous year's branches is reduced is expected to affect oral generation. Third, pine trees are extremely positive and Pine pollen is related to the time of the northeast wind. Therefore, it is believed that it would be good to be located in the northern direction, where the sunlight is good, in an outdoor space. In addition, it is important to use vaginal consumer products in spring and carry out a quarrel involving Mg and Fe during fall to reduce the effect of the Pine pollen, which is an outdoor plant. This is an important part of maintaining and managing pine trees in outdoor spaces as well as the sex determination of pine trees. This study suggested that plant planning, which derives the correlation between pine inorganic element content on sexual determination and takes into account the physiological characteristics of pine trees, is an important issue in the creation of outdoor space. Follow-up research on other factors affecting pine tree sex determination is expected.
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