• Journal of Korean Society of Forest Science
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• v.45 no.1
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• pp.62-67
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• 1979

Ultraviolet microscopy of ultrathin sections of wood has proved to be one of the useful means for determining the lignin distribution in the various regions of the cell wall. Also, spectral approach and quantitative analysis of isolated compound middle lamella fraction from birch xylem have revealed that the lignin associated with the vessel secondary wall and middle lamella is composed predominantly of gualacylpropane units. Lignin deposited in the fiber and ray parenchyma secondary walls is composed mostly of syringylpropane units. The middle lamella lignin around fibers and ray cells contains both guaiacyl and syringyl propane quits. On the basis of the results above, this research was carried out to clarify the origin of milled wood lignin (MWL) by analysing the chemical characteristics of ML MWLs extracted at various milling stages. The amount of phenolic hydroxyl-, ${\alpha}$-carbonyl-, and methoxyl-group in the MWL's increases the milling time. And progressive mining contributes to the merease of ratio of syringylaldehyde to vanillin(S/V ratio) after nitrobenzene oxidation of MWL. Accordingly, It could be concluded that milled wood lignin extracted at the initial milling stage derives from compound middle lamella region of cell wall, whereas, with progressive milling, lignin of secondary wall of fiber is introduced gradually to milled wood lignin. These results are suggesting that heterogeneous chemical structure of lignins in hardwood exists. Although milled wood lignin at the initial stage seems to have lower molecular weight in comparison with milled wood lignin extracted at final milling stage from the result of Gel-filtration curves, further study would be required on molecular weight distribution of milled wood lignin in future.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.30-34
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• 2018

Niemann Pick type C disease (NPC) is an inherited progressive neurodegenerative disorder, due to defects of intracellular lipid trafficking and storage. Hepatosplenomegaly may prevail, while progressive neurodegenerative symptoms such as cerebellar involvement, dystonia, vertical supranuclear ophthalmoplegia, cataplexy, and eventually seizures starting at juvenile or late infantile period may accompany after normal early development. Here we describe a 3-year-old Korean boy with NPC who presented with splenomegaly at age 3. Liver biopsy showed characteristic foamy cell stained by periodic acid-schiff, and molecular analysis for NPC1 identified the compound heterozygous mutations, novel mutation of c.1631G>A (p.Trp544Ter) and c.2662C>T (p. Pro888Ser) as a known mutation. Filipin was strongly stained with unesterified cellular cholesterol in the patient's skin fibroblasts. The patient has received migulstat since age 3 years and his long-term outcome is needed to be observed.

• Journal of Animal Science and Technology
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• v.56 no.8
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• pp.33.1-33.8
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• 2014

Background: Sox 9 is a major marker of chondrocyte differentiation. When chondrocytes are cultured in vitro they progressively de-differentiate and this is associated with a decline in Sox 9 expression. The active form of vitamin D, 1, 25 $(OH)_2D_3$ has been shown to be protective of cartilage in both humans and animals. In this study equine articular chondrocytes were grown in culture and the effects of 1, 25 $(OH)_2D_3$ upon Sox 9 expression examined. The expression of the transient receptor potential vanilloid (TRPV) ion channels 5 and 6 in equine chondrocytes in vitro, we have previously shown, is inversely correlated with de-differentiation. The expression of these channels in response to 1, 25 $(OH)_2D_3$ administration was therefore also examined. Results: The active form of vitamin D (1, 25 $(OH)_2D_3$ when administered to cultured equine chondrocytes at two different concentrations significantly increased the expression of Sox 9 at both. In contrast 1, 25 $(OH)_2D_3$ had no significant effect upon the expression of either TRPV 5 or 6 at either the protein or the mRNA level. Conclusions: The increased expression of Sox 9, in equine articular chondrocytes in vitro, in response to the active form of vitamin D suggests that this compound could be utilized to inhibit the progressive de-differentiation that is normally observed in these cells. It is also supportive of previous studies indicating that $1{\alpha}$, 25-dihydroxyvitamin $D_3$ can have a protective effect upon cartilage in animals in vivo. The previously observed correlation between the degree of differentiation and the expression levels of TRPV 5/6 had suggested that these ion channels may have a direct involvement in, or be modulated by, the differentiation process in vitro. The data in the present study do not support this.

• 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 Pharmacopuncture
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• v.21 no.4
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• pp.277-283
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• 2018

Objectives: Ethanol withdrawal following its chronic use is a serious outcome and challenging to treatment. The chronic use of ethanol induces a progressive neuroplasticity in different reigns of brain. In this study we evaluated the effects of aqueous extract of Crocus sativus L. (saffron) and its active compound, crocin, on the withdrawal behavior induced after repeated administration of ethanol, in two regimens of prophylactic (administration of drugs concomitant with the induction of dependence) and treatment (administration of drugs during the period of ethanol withdrawal) in mice which received ethanol. Methods: Ethanol dependence was induced by oral administration of 10% v/v ethanol (2 g/kg) for 7 days. The aqueous extracts of saffron (40, 80 and 160) and crocin (10, 20 and 40 mg/kg) were administered to mice in two regimens of prophylactic (along with ethanol) and treatment (during withdrawal period). Diazepam (1 mg/kg) was used as a positive control. Six hours after discontinuation of the ethanol, seizure was evaluated by the sub-convulsive dose of pentyleneltetrazole (PTZ) (30 mg/kg). The open field test and Rota rod test were used for evaluation of locomotor activity and motor incoordination, respectively. Results: Both extracts and crocin increased the number of crossed lined in the open field test. PTZ kindling seizure was inhibited in animals received extract (80 and 160 mg/kg) in both regimens. Motor incoordination was only improved following administration of crocin. Conclusion: The aqueous extract of saffron and crocin can be considered as safe agents and reliable alternative to diazepam in management of ethanol withdrawal syndrome.

• Biomolecules & Therapeutics
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• v.27 no.2
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• pp.178-184
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• 2019

Parkinson's disease is a neurodegenerative disease characterized by the progressive loss of dopaminergic neurons within the substantia nigra pars compacta. In the present study, we investigated whether ${\beta}-Lapachone$ (${\beta}-LAP$), a natural naphthoquinone compound isolated from the lapacho tree (Tabebuia avellanedae), elicits neuroprotective effects in a 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced Parkinson's disease mouse model. ${\beta}-LAP$ reduced the tyrosine hydroxylase (TH)-immunoreactive fiber loss induced by MPTP in the dorsolateral striatum, and alleviated motor dysfunction as determined by the rotarod test. In addition, ${\beta}-LAP$ protected against MPTP-induced loss of TH positive neurons, and upregulated B-cell lymphoma 2 protein (Bcl-2) expression in the substantia nigra. Based on previous reports on the neuroprotective role of nuclear factor-E2-related factor-2 (Nrf2) in neurodegenerative diseases, we investigated whether ${\beta}-LAP$ induces upregulation of the Nrf2-hemeoxygenae-1 (HO-1) signaling pathway molecules in MPTP-injected mouse brains. Western blot and immunohistochemical analyses indicated that ${\beta}-LAP$ increased HO-1 expression in glial fibrillary acidic protein-positive astrocytes. Moreover, ${\beta}-LAP$ increased the nuclear translocation and DNA binding activity of Nrf2, and the phosphorylation of upstream adenosine monophosphate-activated protein kinase (AMPK). ${\beta}-LAP$ also increased the localization of p-AMPK and Nrf2 in astrocytes. Collectively, our data suggest that ${\beta}-LAP$ exerts neuroprotective effect in MPTP-injected mice by upregulating the p-AMPK/Nrf2/HO-1 signaling pathways in astrocytes.

• Journal of Genetic Medicine
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• v.19 no.1
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• pp.22-26
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• 2022

Cerebrotendinous xanthomatosis (CTX) is a rare genetic disease caused by a deficiency of enzymes for the synthesis of bile acid, resulting in the accumulation of cholestanol with reduced chenodeoxycholic acid (CDCA) production and causing various symptoms such as chronic diarrhea in infancy, juvenile cataracts in childhood, tendon xanthomas in adolescence and young adulthood, and progressive neurologic dysfunction in adulthood. Because oral CDCA replacement therapy can effectively prevent disease progression, early diagnosis and treatment are critical in CTX. This study reports the case of CTX in a 10-year-old male who presented with Achilles tendon xanthoma and mild intellectual disability. Biochemical testing showed normal cholesterol and sitosterol levels but elevated cholestanol levels. Genetic testing showed compound heterozygous variants of CYP27A1, c.379C>T (p.Arg127Trp), and c.1214G>A (p.Arg405Gln), which confirmed the diagnosis of CTX. The patient had neither cataracts nor other focal neurologic deficits and showed no abnormalities on brain imaging. The patient received oral CDCA replacement therapy without any adverse effects; thereafter, the cholestanol level decreased and no disease progression was noted. The diagnostic possibility of CTX should be considered in patients with tendon xanthoma and normolipidemic conditions to prevent neurological deterioration.

• Applied Microscopy
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• v.39 no.2
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• pp.107-115
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• 2009

The programed cell death of the cutaneous epithelial tissue during tail regression stages in anuran tadpoles of the blackspotted frog, Rana nigromaculata were visualized by the histochemical and transmission electron microscopic techniques. Metamorphotic changes in the tail regression during the period of the Shumway stage number 31 to 33 are characterized by the disappearance of mucous layer and formation of compound epithelium through cutaneous thickening. Following the TUNEL (terminal deoxynucleotidyl transferase-mediated biotinylated d-uridine triphosphate nick end labeling) staining technique, the apoptotic cells were detected at the distal region of the tail skin initially, but they can be seen at the proximal region according to their following development. It has been also revealed that the number of the TUNEL-positive cells gradually increased from apical to basal direction of the epithelial layers during the tail regressing stages. Following the TEM observation, the early apoptotic cells shown in the epithelium demonstrated condensation and margination of the chromatin material at the nuclear periphery. Another epithelial apoptotic cells were shown nuclear fragmentation, membrane blebbing and cytoplasmic condensation. Following the process of the apoptotic degradation, well preserved organelles and nuclear fragments can be identified in the cytoplasm of lysosome-rich cells, however they soon reduced to lysosomal residual bodies through the progressive degradation.

• Journal of Korean Society of Coastal and Ocean Engineers
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• v.27 no.4
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• pp.246-257
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• 2015

The progressive degradation paths of structures have quantitatively been tracked by using stochastic processes, such as Wiener process, gamma process and compound Poisson process, in order to consider both the sampling uncertainty due to the usual lack of damage data and the temporal uncertainty associated with the deterioration evolution. Several important features of stochastic processes which should carefully be considered in application of the stochastic processes to practical problems have been figured out through assessing cumulative damage and lifetime distribution as a function of time. Especially, the Wiener process and the gamma process have straightforwardly been applied to armors of rubble-mound breakwaters by the aid of a sample path method based on Melby's formula which can estimate cumulative damage levels of armors over time. The sample path method have been developed to calibrate the related-parameters required in the stochastic modelling of armors of rubble-mound breakwaters. From the analyses, it is found that cumulative damage levels of armors have surely been saturated with time. Also, the exponent of power law in time, that plays a significant role in predicting the cumulative damage levels over time, can easily be determined, which makes the stochastic models possible to track the cumulative damage levels of armors of rubble-mound breakwaters over time. Finally, failure probabilities with respect to various critical limits have been analyzed throughout its anticipated service life.

• Journal of the Korean Society of Marine Environment & Safety
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• v.15 no.1
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• pp.1-9
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• 2009

Distribution characteristics of organophosphorous pesticides (OPs) were studied over the period from May to September, 2006 in Asan Bay, Korea. During the study period, 28 kinds of organophosphorous pesticides dissolved in surface water and adsorbed on suspended particle were measured. In the surface water, the dominant OPs were IBP and DDVP, and the concentration were in the ranges from not detected to 2014.4 ng/L for IBP and 3.2 to 696.3 ng/L for DDVP. The highest concentrations of OPs in the surface waters in Asan Bay appeared in July and August showing that seasonal distributions of OPs depend on application time and precipitation. The concentrations of OPs generally decreased with the increase of distance from the mouth of Asan Bay, implying progressive dilution of pesticides in the estuarine system. OPs residue in Asan Bay was relatively higher than in other area of Korea. The concentrations of the observed OPs concentrations did not exceed the seawater quality standard of Korea. DDVP, IBP, Diazinon, Phorate, Azinphos ethyl and Chlorfenvinfos had higher adsorption capacity onto suspended particle than the other OPs. In the study area, adsorption coefficients $(K_d)$ of OPs were closely related to the Log $K_ow$ of each compound.