• Journal of Korean Medical Science
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• v.33 no.51
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• pp.324.1-324.6
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• 2018

Oxysterol $7{\alpha}$-hydroxylase deficiency is a very rare liver disease categorized as inborn errors of bile acid synthesis, caused by CYP7B1 mutations. As it may cause rapid progression to end-stage liver disease even in early infancy, a high index of suspicion is required to prevent fatal outcomes. We describe the case of a 3-month-old boy with progressive cholestatic hepatitis and severe hepatic fibrosis. After excluding other etiologies for his early liver failure, we found that he had profuse urinary excretion of $3{\beta}$-monohydroxy-${\Delta}^5$-bile acid derivatives by gas chromatography/mass spectrometry analysis with dried urine spots on filter paper. He was confirmed to have a compound heterozygous mutation (p.Arg388Ter and p.Tyr469IlefsX5) of the CYP7B1 gene. After undergoing liver transplantation (LT) from his mother at 4 months of age, his deteriorated liver function completely normalized, and he had normal growth and development until the current follow-up at 33 months of age. We report the first Korean case of oxysterol $7{\alpha}$-hydroxylase deficiency in the youngest infant reported to undergo successful living donor LT to date.

• Journal of the Geological Society of Korea
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• v.54 no.5
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• pp.579-584
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• 2018

It is crucial to determine a mixing ratio using an end-member mixing analysis when there is seawater intrusion. In this study, an error from the calculation of the mixing ratio between seawater and freshwater based on the principles of uncertainty was determined. I present the errors in the calculated mixing ratios as a function of the chemical difference between the mean seawater concentrations and standard deviations. The error is caused by: (1) the mixing ratio between seawater and freshwater; (2) the difference between the mean concentration and the standard deviation; and (3) the difference of the tracer concentration between freshwater and seawater (inversely). In particular, the error may determine hydrogeochemical process (either precipitation or dissolution) when a value of ionic delta (difference between measured and theoretical concentration) is close to zero during cation exchange by seawater intrusion.

• BMB Reports
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• v.52 no.11
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• pp.641-646
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• 2019

The Ron proto-oncogene is a human receptor for macrophage-stimulating protein (MSP). The exclusion of exon 11 in alternative splicing generates ${\Delta}RON$ protein that is constitutively activated. Heterogenous ribonucleaoprotein (hnRNP) $C_1/C_2$ is one of the most abundant proteins in cells. In this manuscript, we showed that both hnRNP $C_1$ and $C_2$ promoted exon 11 inclusion of Ron pre-mRNA and that hnRNP $C_1$ and hnRNP $C_2$ functioned independently but not cooperatively. Moreover, hnRNP $C_1$ stimulated exon 11 splicing through intron 10 activation but not through intron 11 splicing. Furthermore, we showed that, whereas the RRM domain was required for hnRNP $C_1$ function, the Asp/Glu domain was not. In conclusion, hnRNP $C_1/C_2$ promoted exon 11 splicing independently by stimulating intron 10 splicing through RRM but not through the Asp/Glu domain.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.22 no.5
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• pp.561-566
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• 2021

The optimum kernel size for error-distribution estimation with given error samples cannot be used in the weight adjustment of minimum Euclidean distance between error distributions (MED) algorithms. In this paper, a new adaptive kernel estimation method for convergence enhancement of MED algorithms is proposed. The proposed method uses the average rate of change in error power with respect to a small interval of the kernel width for weight adjustment of the MED learning algorithm. The proposed kernel adjustment method is applied to experiments in communication channel compensation, and performance improvement is demonstrated. Unlike the conventional method yielding a very small kernel calculated through optimum estimation of error distribution, the proposed method converges to an appropriate kernel size for weight adjustment of the MED algorithm. The experimental results confirm that the proposed kernel estimation method for MED can be considered a method that can solve the sensitivity problem from choosing an appropriate kernel size for the MED algorithm.

• Development and Reproduction
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• v.24 no.4
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• pp.287-296
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• 2020

The absence of functional estrogen receptor α (Esr1) results in an overgrowth of the epididymal fat, as observed in estrogen receptor α knockout (ERαKO) mouse. The present research was aimed to evaluate expression of various molecules associated with adipocyte differentiation and maturation in the epididymal fat of ERαKO mouse at several postnatal ages by using quantitative real-time polymerase chain reaction. The highest transcript levels of all molecules were detected at 12 months of postnatal age, except leptin which the mRNA level was increased at 5 months of age and was unchanged until 12 months of age. The expression levels of CCAAT enhancer binding protein (Cebp) alpha, androgen receptor, and lipoprotein lipase were decreased at 5 months of age but increased at about 8 months of age. The mRNA levels of Cebp gamma and sterol regulatory element binding transcription factor 1 remained steady until 8 months of age. Continuous increases of transcript levels during postnatal period were found in Cebp beta, estrogen receptor (ER) beta, fatty acid binding protein 4, and delta like non-canonical Notch ligand 1. The increases of peroxisome proliferator-activated receptor gamma and adiponectin mRNA levels were detected as early as 8 months of age. The levels of fatty acid synthase and resistin transcript at 5 and 8 months of age were lower than that at 2 months of age. These findings show the aberrant expression patterns of genes related to adipocyte differentiation and maturation in the postnatal epididymal fat pad by the disruption of ER alpha function.

• International Journal of Stem Cells
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• v.16 no.1
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• pp.108-116
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• 2023

γδ T cells are a rare and unique prototype of T cells that share properties with natural killer cells in secondary lymphoid organs. Although many studies have revealed the function and importance of adult-derived γδ T cells in cancer biology and regenerative medicine, the low numbers of these cells hamper their application as therapeutic cell sources in the clinic. To solve this problem, pluripotent stem cell-derived γδ T cells are considered alternative cell sources; however, few studies have reported the generation of human pluripotent stem cell-derived γδ T cells. In the present study, we investigated whether lymphoid lineage γδ T cells were successfully generated from human pluripotent stem cells via hemogenic endothelium under defined culture conditions. Our results revealed that pluripotent stem cells successfully generated γδ T cells with an overall increase in transcriptional activity of lymphoid lineage genes and cytolytic factors, indicating the importance of the optimization of culture conditions in generating lymphoid lineage γδ T cells. We uncovered an initial step in differentiating γδ T cells that could be applied to basic and translational investigations in the field of cancer biology. Based on our result, we will develop an appropriate method to purify γδ T cells with functionality and it helpful for the study of basic mechanism of γδ T cells in pathophysiologic condition as well as clinic application.

• Journal of Radiation Protection and Research
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• v.33 no.1
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• pp.13-20
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• 2008

The flow rate measurements in a multi-phase flow pipeline were evaluated quantitatively by means of a clamp-on sealed radioisotope based on a cross correlation signal processing technique. The flow rates were calculated by a determination of the transit time between two sealed gamma sources by using a cross correlation function following FFT filtering, then corrected with vapor fraction in the pipeline which was measured by the ${\gamma}$-ray attenuation method. The pipeline model was manufactured by acrylic resin(ID. 8 cm, L=3.5 m, t=10 mm), and the multi-phase flow patterns were realized by an injection of compressed $N_2$ gas. Two sealed gamma sources of $^{137}Cs$ (E=0.662 MeV, ${\Gamma}$ $factor=0.326\;R{\cdot}h^{-1}{\cdot}m^2{\cdot}Ci^{-1}$) of 20 mCi and 17 mCi, and radiation detectors of $2"{\times}2"$ NaI(Tl) scintillation counter (Eberline, SP-3) were used for this study. Under the given conditions(the distance between two sources: 4D(D; inner diameter), N/S ratio: $0.12{\sim}0.15$, sampling time ${\Delta}t$: 4msec), the measured flow rates showed the maximum. relative error of 1.7 % when compared to the real ones through the vapor content corrections($6.1\;%{\sim}9.2\;%$). From a subsequent experiment, it was proven that the closer the distance between the two sealed sources is, the more precise the measured flow rates are. Provided additional studies related to the selection of radioisotopes their activity, and an optimization of the experimental geometry are carried out, it is anticipated that a radioisotope application for flow rate measurements can be used as an important tool for monitoring multi-phase facilities belonging to petrochemical and refinery industries and contributes economically in the light of maintenance and control of them.

• Journal of Yeungnam Medical Science
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• v.23 no.1
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• pp.52-61
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• 2006

Background: Korean ginseng (KG) has been used as a general tonic, and for voiding dysfunction for a long time in oriental society. However, scientific basic studies on the use of KG, have been rare, especially for voiding and erectile dysfunction. This study was performed to investigate the effects of KG on voiding and erectile function by examining the effects of total saponin (TS) on the bladder, urethral and penile cavernosal smooth muscle. Materials and methods: To examine the effects of TS, NewZeland white rabbits were used to obtain tissue strips from the smooth muscle of the bladder, proximal urethra and corpus cavernosum. Adult Sprague Dawley rats were used to examine the changes in urodynamic findings and penile erection after administration of TS. Results: In proximal urethral strips, the rate of relaxation of the proximal urethra was increased from $9.0{\pm}2.9$ to $33.7{\pm}4.8%$ in a dose-dependent manner when the concentration of TS was added accumulatively from 0.25 mg/ml to 4.0 mg/ml (p<0.05). However, no significant response was observed in the bladder strips within these concentration ranges. For the corpus cavernosal strips, the rate of relaxation ranged from $5.8{\pm}2.1$ to $36.7{\pm}5.8%$, increasing in a dose-dependent manner when TS was increased from 1.0 mg/ml to 4.0 mg/ml (p<0.05). After administration of 0.1 ml of TS (32 mg/ml) in the rat, the bladder pressure was $37.5{\pm}8.5$ mmHg at $52.1{\pm}7.0$ sec. during isovolumetric bladder contraction, showing no significant differences from $35.7{\pm}7.8mmHg$ and $50.7{\pm}7.2$ sec, respectively, before treatment. However, when 0.1 ml of TS (32 mg/ml) was administered, the relative reduction of urethral pressure was $6.9{\pm}0.5mmHg$ at $62{\pm}7.5$ sec, which was significantly higher compared to $4.6{\pm}1.1mmHg$ at $45{\pm}10$ sec before treatment (p<0.05). For the cavernosal injection study, the change in intracavernosal pressure (${\Delta}ICP$) was examined after administering 0.1 ml of TS. The cumulative additions of TS at concentrations from 0.5 mg/ml to 32 mg/ml increased ${\Delta}ICP$ from $1.3{\pm}0.5$ to $21.3{\pm}7.8mmHg$ in a dose-dependent manner (p<0.05). The duration of tumescence was from $0.3{\pm}0.1$ to $5.2{\pm}0.2$ min, showing dose-dependent increase (p<0.05). Furthermore, the cumulative addition of TS at concentrations from 0.5 mg/ml upto 32 mg/ml did not cause any significant change in systemic blood pressure. Conclusion: These results suggest that ginseng improves voiding functions, which is mainly achieved by TS relaxing the proximal urethra, the most important part of the bladder outlet function. In addition, ginseng safely induced a penile erection hemodynamically by relaxing the corpus cavernosum.

• Tuberculosis and Respiratory Diseases
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• v.45 no.1
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• pp.153-168
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• 1998

Background: The existing data indicate that obstructive sleep apnea syndrome contributes to the development of cardiovascular dysfunction such as systemic hypertension and cardiac arrhythmias, and the cardiovascular dysfunction has a major effect on high long-term mortality rate in obstructive sleep apnea syndrome patients. To a large extent the various studies have helped to clarify the pathophysiology of obstructive sleep apnea, but many basic questions still remain unanswered. Methods: In this study, the influence of obstructive sleep apnea on systemic blood pressure, cardiac rhythm and urinary catecholamines concentration was evaluated. Over-night polysomnography, 24-hour ambulatory blood pressure and ECG monitoring, and measurement of urinary catecholamines, norepinephrine (UNE) and epinephrine (UEP), during waking and sleep were undertaken in obstructive sleep apnea syndrome patients group (OSAS, n=29) and control group (Control, n=25). Results: 1) In OSAS and Control, UNE and UEP concentrations during sleep were significantly lower than during waking (P<0.01). In UNE concentrations during sleep, OSAS showed higher levels compare to Control (P<0.05). 2) In OSAS, there was a increasing tendency of the number of non-dipper of nocturnal blood pressure compare to Control (P=0.089). 3) In both group (n=54), mean systolic blood pressure during waking and sleep showed significant correlation with polysomnographic data including apnea index (AI), apnea-hypopnea index (AHI), arterial oxygen saturation nadir ($SaO_2$ nadir) and degree of oxygen desaturation (DOD). And UNE concentrations during sleep were correlated with AI, AHI, $SaO_2$ nadir, DOD and mean diastolic blood pressure during sleep. 4) In OSAS with AI>20 (n==14), there was a significant difference of heart rates before, during and after apneic events (P<0.01), and these changes of heart rates were correlated with the duration of apnea (P<0.01). The difference of heart rates between apneic and postapneic period (${\Delta}HR$) was significantly correlated with the difference of arterial oxygen saturation between before and after apneic event (${\Delta}SaO_2$) (r=0.223, P<0.001). 5) There was no significant difference in the incidence of cardiac arrhythmias between OSAS and Control In Control, the incidence of ventricular ectopy during sleep was significantly lower than during waking. But in OSAS, there was no difference between during waking and sleep. Conclusion : These results suggested that recurrent hypoxia and arousals from sleep in patients with obstructive sleep apnea syndrome may increase sympathetic nervous system activity, and recurrent hypoxia and increased sympathetic nervous system activity could contribute to the development of cardiovascular dysfunction including the changes of systemic blood pressure and cardiac function.

• Journal of the Korean Crystal Growth and Crystal Technology
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• v.28 no.5
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• pp.179-184
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• 2018

To lessen oxygen concentrations in a wafer through modifying the length of graphite heaters, we investigated the influence of relative distance from heater to quartz crucible on temperature profile of hot-zone in Czochralski silicon-crystal growth by simulation. In particular, ATC temperature and power profiles as a function of different ingot body positions were investigated for five different heater designs; (a) typical side heater (SH), (b) short side heater-up (SSH-up), (c) short side heater-low (SSH-low), (d) bottom heater without side heater (Only-BH), and (e) side heater with bottom heater (SH + BH). It was confirmed that lower short side heater exhibited the highest ATC temperature, which was attributed to the longest distance from triple point to heater center. In addition, for the viewpoint of energy efficiency, it was observed that the typical side heater showed the lowest power because it heated more area of quartz crucible than that of others. This result provides the possibility to predict the feed-forward delta temperature profile as a function of various heater designs.