• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.141-147
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• 2016

Hypophosphatasia is caused by the mutations in ALPL, which encodes tissue-nonspecific alkaline phosphatase (TNSALP). It can be inherited either in an autosomal dominant or recessive manner. Clinically, hypophophosphatasia is characterized by skeletal findings similar to those in rickets or osteomalacia, but serum alkaline phosphatase levels are decreased in the affected patients. Hypophosphatasia can be classified into six clinical forms according to age at diagnosis and severity of symptoms: perinatal lethal, infantile, childhood, adult, odontohypophosphatasia, and perinatal benign. As being a very rare disease, only one case has been reported in Korean population. Here we describe a case with perinatal benign hypophosphatasia with recessive ALPL mutations. Bowing of lower legs was detected in prenatal period and low serum alkaline phosphatase level was noted after birth. During the follow-up evaluation for 4.5 years, bone mineralization and legs bowing were improved but the growth retardation was persistent. As the recombinant bone-targeted human TNSALP became available, the clinical improvement of the affected patients is expected including the case described here with this treatment. More efforts are needed to identify the cases affected by hypophosphatasia.

• Korean Journal of Food Science and Technology
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• v.30 no.5
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• pp.1018-1023
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• 1998

Hydrocarbons in dry soup base and its ingredients in instant noodle were analyzed to determine whether the analysis of hydrocarbons is a potential method to detect post-irradiation of the soup base. Soup base ingredients were irradiated individually, irradiated before mixing, or mixed after irradiation. Lipids were extracted with hexane and hydrocarbons were separated from the lipids through Florisil column. The hydrocarbons were analyzed with GC. Hydrocarbons C17:2, C16:3, C17:1, and C16:2 were detected in palm oil, red pepper powder, and sesame seeds irradiated at 10 kGy, but not in unirradiated ones. C17:2, C16:3, C17:1, and C16:2 were not detected in the soup base mixture of unirradiated ingredients. The four hydrocarbons were detected in the soup base mixture using irradiated palm oil or sesame seeds. In the mixture using irradiated red pepper powder, C17:2 and C16:3 were detected. When the soup mixture was irradiated after mixing unirradiated ingredients, C17:2, C17:1, and C16:2 were detected in the sample irradiated at 1 kGy, and C17:2, C16:3, C17:1, and C16:2 were in large amounts at 5 and 10 kGy.

• Biomolecules & Therapeutics
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• v.26 no.2
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• pp.146-156
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• 2018

Spermidine is a naturally occurring polyamine compound that has recently emerged with anti-aging properties and suppresses inflammation and oxidation. However, its mechanisms of action on anti-inflammatory and antioxidant effects have not been fully elucidated. In this study, the potential of spermidine for reducing pro-inflammatory and oxidative effects in lipopolysaccharide (LPS)-stimulated macrophages and zebrafish was explored. Our data indicate that spermidine significantly inhibited the production of pro-inflammatory mediators such as nitric oxide (NO) and prostaglandin $E_2$ ($PGE_2$), and cytokines including tumor necrosis $factor-{\alpha}$ and $interleukin-1{\beta}$ in RAW 264.7 macrophages without any significant cytotoxicity. The protective effects of spermidine accompanied by a marked suppression in their regulatory gene expression at the transcription levels. Spermidine also attenuated the nuclear translocation of $NF-{\kappa}B$ p65 subunit and reduced LPS-induced intracellular accumulation of reactive oxygen species (ROS) in RAW 264.7 macrophages. Moreover, spermidine prevented the LPS-induced NO production and ROS accumulation in zebrafish larvae and was found to be associated with a diminished recruitment of neutrophils and macrophages. Although more work is needed to fully understand the critical role of spermidine on the inhibition of inflammation-associated migration of immune cells, our findings clearly demonstrate that spermidine may be a potential therapeutic intervention for the treatment of inflammatory and oxidative disorders.

• Korean Journal of Environmental Biology
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• v.17 no.2
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• pp.129-138
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• 1999

Chlorinated aromatic compounds are one of the largest groups of environmental pollutants as a result of world-wide distribution by using them as herbicides, insecticides, fungicides, solvents, hydraulic and heat transfer fluids, plasticizers, and intermediates for chemical synthesis. Because of their toxicity, persistence, and bioaccumulation, the compounds contaminated ubiquitously in the biosphere has attracted public concerns in terms of serious influences to wild lives and a human being, such as carcinogenicity, mutagenicity, and disturbance in endocrine systems. The biological recalcitrance of the compounds is caused by the number, type, and position of the chlorine substituents as well as by their aromatic structures. In general, the carbon-halogen bonds increase the recalcitrance by increasing electronegativity of the substituent, so that the dechlorination of the compounds is focused as an important mechanism for biodegradation of chlorinated aromatics, along with the cleavage of aromatic rings. The removal of the chlorine substituents has been known as a key step for degradation of chlorinated aromatic compounds under aerobic condition. This can occur as an initial step via oxygenolytic, reductive, and hydrolytic mechanisms. The studies on the biochemistry and genetics about microbial dechlorination give us the potential informations for microbial degradation of xenobiotics contaminated in natural microcosms. Such investigations might provide biotechnological approaches to solve the environmental contamination, such as designing effective bioremediation systems using genetically engineered microorganisms.

• Applied Microscopy
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• v.32 no.3
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• pp.285-290
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• 2002

The therapeutic effect of safflower seed powder on experimental osteoporosis in the rat induced by ovariectomy was evaluated. Thirty Sprague-Dawley rats were ovariectomized at the age of 12 weeks. Seven weeks postovariectomy, rats were divided into two groups: control and safflower seed powder treated group. Five animals from each group were sacrificed at the following time points: 1, 3, and 5 weeks. Scanning electron microscopic observation and morphometric analysis of the tibiae epiphysis showed that the administration of safflower seed powder significantly prevented reduction of cortical bone width and bone volume compared with the control group. In conclusion, safflower seed powder contains something that prevent bone loss due to estrogen deficiency, and was effective in preventing the osteoporotic decrease of bone mass.

• Journal of Genetic Medicine
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• v.12 no.1
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• pp.25-30
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• 2015

Purpose: Charcot-Marie-Tooth disease (CMT) is a peripheral neuropathy mainly divided into CMT type 1 (CMT1) and CMT2 according to the phenotype and genotype. Although molecular pathologies for each genetic causative have not been revealed in CMT2, the correlation between cell death and accumulation of misfolded proteins in the endoplasmic reticulum (ER) of Schwann cells is well documented in CMT1. Establishment of in vitro models of ER stress-mediated Schwann cell death might be useful in developing drug-screening systems for the treatment of CMT1. Materials and Methods: To develop high-throughput screening (HTS) systems for CMT1, we generated cell models using transient expression of mutant proteins and chemical induction. Results: Overexpression of wild type and mutant peripheral myelin protein 22 (PMP22) induced ER stress. Similar results were obtained from mutant myelin protein zero (MPZ) proteins. Protein localization revealed that expressed mutant PMP22 and MPZ proteins accumulated in the ER of Schwann cells. Overexpression of wild type and L16P mutant PMP22 also reduced cell viability, implying protein accumulation-mediated ER stress causes cell death. To develop more stable screening systems, we mimicked the ER stress-mediated cell death in Schwann cells using ER stress inducing chemicals. Thapsigargin treatment caused cell death via ER stress in a dose dependent manner, which was measured by expression of ER stress markers. Conclusion: We have developed genetically and chemically induced ER stress models using Schwann cells. Application of these models to HTS systems might facilitate the elucidation of molecular pathology and development of therapeutic options for CMT1.

• Applied Biological Chemistry
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• v.42 no.1
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• pp.29-33
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• 1999

To investigate the effects of Angelica gigas Nakai diet on lipid metabolism, alcohol metabolism and liver function of rats administered with chronic ethanol, Sprague-Dawley male rats were fed either AIN-76 diet (control), control diet with ethanol, control plus Angelica gigas Nakai diet, or control plus Angelica gigas Nakai diet with ethanol for 30 days. On the 21st day, all of the rats were given an oral dose of ethanol and blood-ethanol concentration was monitored for the next 5 hours. The results obtained were: 1) Upon ethanol administration, the blood ethanol concentration was decreased from 2 hr significantly in the group of control plus Angelica gigas Nakai diet compared with control diet group; 2) The blood ethanol oxidation rate was increased in the group of control plus Angelica gigas Nakai diet with ethanol compared with control diet group or control plus Angelica gigas Nakai diet group. After 30 days, rats were sacrificed and then lipid and enzyme determinations in blood and liver were carried out. The results obtained were: 1) LDL-cholesterol in the blood of control plus Angelica gigas Nakai diet group was decreased significantly compared with control diet group; 2) Angelica gigas Nakai diet decreased liver triglyceride and total lipid and blood ${\gamma}-GTP$ level increased due to the chronic ethanol administration. These data suggest that Angelica gigas Nakai can have a recovery function on the symptoms of alcohol related diseases.

• Journal of Environmental Science International
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• v.25 no.10
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• pp.1349-1368
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• 2016

Concentrations of fine particulate matter ($PM_{2.5}$) and several of its particle constituents measured outside homes in Houston, Texas, and Los Angeles, California, were characterized using multiple regression analysis with proximity to point and mobile sources and meteorological factors as the independent variables. $PM_{2.5}$ mass and the concentrations of organic carbon (OC), elemental carbon (EC), benzo-[a]-pyrene (BaP), perylene (Per), benzo-[g,h,i]-perylene (BghiP), and coronene (Cor) were examined. Negative associations of wind speed with concentrations demonstrated the effect of dilution by high wind speed. Atmospheric stability increase was associated with concentration increase. Petrochemical source proximity was included in the EC model in Houston. Area source proximity was not selected for any of the $PM_{2.5}$ constituents' regression models. When the median values of the meteorological factors were used and the proximity to sources varied, the air concentrations calculated using the models for the eleven $PM_{2.5}$ constituents outside the homes closest to influential highways were 1.5-15.8 fold higher than those outside homes furthest from the highway emission sources. When the median distance to the sources was used in the models, the concentrations of the $PM_{2.5}$ constituents varied 2 to 82 fold, as the meteorological conditions varied over the observed range. We found different relationships between the two urban areas, illustrating the unique nature of urban sources and suggesting that localized sources need to be evaluated carefully to understand their potential contributions to $PM_{2.5}$ mass and its particle constituents concentrations near residences, which influence baseline indoor air concentrations and personal exposures. The results of this study could assist in the appropriate design of monitoring networks for community-level sampling and help improve the accuracy of exposure models linking emission sources with estimated pollutant concentrations at the residential level.

• Clinical and Experimental Reproductive Medicine
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• v.38 no.3
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• pp.168-173
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• 2011

Objective: To examine the association between methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism and hyperhomocysteinemia in women with unexplained recurrent miscarriages (RM) and to investigate the association between MTHFR genotype variants and alloimmune activation, proportion of peripheral blood natural killer (pbNK) cells. Methods: A total of 39 patients with a history of two or more unexplained miscarriages were recruited to this study. The controls were women who had a live birth without a history of RM (n=50). The proportion of pbNK cells was measured by flow cytometry. Plasma homocysteine levels and the incidence of the MTHFR variant of the RM and control groups were compared. The proportion of pbNK cells was compared to the MTHFR variants in the RM group. Results: No differences were found between the two groups' mean plasma homocysteine levels ($7.6{\pm}1.5{\mu}mol$/L vs. $7.1{\pm}2.1{\mu}mol$/L) or incidence of the MTHFR genotype variant (CC, 35% vs. 33%; CT, 40% vs. 53%; and TT, 25% vs. 14%). In the RM group, individuals with the TT variant ($7.7{\pm}1.1{\mu}mol$/L) had higher homocysteine levels than those with the CC and CT variants ($7.4{\pm}1.9{\mu}mol$/L and $7.4{\pm}1.2{\mu}mol$/L) and those with the CT variant ($19.2{\pm}8.1%$) had a higher proportion of CD3-/CD56+ pbNK cells than those with the CC and TT variants ($17.7{\pm}6.6%$ and $17.9{\pm}7.0%$), but the results of both comparisons were statistically insignificant. Conclusion: These preliminary results show no difference in plasma homocysteine levels between the RM and control groups or among MTHFR genotype variants in the RM group, which may suggest that the plasma homocysteine level is difficult to use as a predictive marker of RM in the Korean population. A study of a larger number of patients is needed.

• Korean Journal of Environment and Ecology
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• v.7 no.2
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• pp.112-117
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• 1994

The present conservation status on the rare and endangered plants in Tokyusan National Park were surveyed and re-evaluated by the new criteria which prepared by I.U.C.N. The species such as Lonicera vidalii, Clematis chiisanensis, Cypripedium macranthum and Allium taquetii were grouped as Critical, Eranthis stellata, Abies koreana, Taxus cuspidata, Tricyrtis dilatata, Paeonia japonaca, Stewartia koreana, Rhododendron tschnoskii, Buplerum euphorbioides, Lilium cernum and Oreorchis patens were grouped as Endangered, Adonis amurenis and Disporum ovale were grouped as Vulnerable. The above mentioned species were mainly located at the vicinity of mountain trails and projected developing sites. The potentials for the habitat destructions, mainly, due to human activities are expected to be serious in the future unless the proper management plans prepared for the specific plants and the specific habitats.