• Childhood Kidney Diseases
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• v.20 no.2
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• pp.83-87
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• 2016

Congenital chloride diarrhea (CLD) is a rare autosomal recessive disease that is difficult to diagnose. CLD requires early treatment to correct electrolyte imbalance and alkalosis and to prevent severe dehydration. Renal injury is clearly associated with defective electrolyte balance induced by CLD, particularly during the first months or years of life. A 7-year-old boy was diagnosed with CLD following detection of a homozygous mutation (c.2063-1G>T) in SLC26A3 at 6 months of age. During treatment with electrolyte supplements, mild proteinuria was detected at 8 months of age, and is still present. Renal biopsy showed the presence of focal renal dysplasia, with metaplastic cartilage and mononuclear cell infiltration, calcification, and fibrosis in the interstitium. Up to two-thirds of the glomeruli exhibited global obsolescence, mostly aggregated in the dysplastic area. In nondysplastic areas, the glomeruli were markedly increased in size and severely hypercellular, with increased mesangial matrix, and displayed segmental sclerosis. The marked glomerular hypertrophy with focal segmental glomerulosclerosis suggested a compensatory reaction to the severe nephron loss or glomerular obsolescence associated with renal dysplasia, with superimposed by CLD aggravating the tubulointerstitial damage.

• BMB Reports
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• v.41 no.4
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• pp.322-327
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• 2008

In this study, we compared the gene expression profiles of non-syndromic hyperplastic dental follicle (HDF) fibroblasts and normal dental follicle (NDF) fibroblasts using cDNA micro-arrays, quantitative PCR, and immunohistochemical staining. Microarray analysis showed that several collagens genes were upregulated in the HDF's, including collagen types I, IV, VIII, and XI and TIMP-1, -3, and -4 (fold ratio > 2.0). In contrast, the expression of MMP-1, -3, -10, and -16 together with IL-8 was more than two fold downregulated. The differential expression of the genes encoding alkaline phosphatase, MMP-1, -3, -8, and IL-8 was confirmed by quantitative RT-PCR, while that of 24 HDFs and 18 NDFs was confirmed by immunohistochemical analysis. However, HDFs showed stronger expression of MMP-3 than NDFs (P < 0.001). Collectively, these results indicate that defective regulation of MMPs mediating connective tissue remodeling may be responsible for abnormal tooth eruption.

• Food Science of Animal Resources
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• v.30 no.4
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• pp.568-574
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• 2010

This study introduced a chick embryos’ infection model to elucidate the pathogenesis mechanism of Pseudomonas aeruginosa, which causes serious diseases in human after ingestion of P. aeruginosa-contaminated animal originated foods. The embryonic chick model is able to give a rapid and relatively inexpensive method to assess bacterial pathogenicity compared to embryos of other vertebrates. Embryos were infected with P. aeruginosa and elastase-deficient P. aeruginosa. After infection with P. aeruginosa cells, total bacterial cell numbers and gelatinase activities in the embryos were compared. Thereafter, precartilage condensation and chondrogenesis were assessed by peanut agglutinin (PNA) binding on day 3 and by Alcian blue staining for sulfated proteoglycans on day 5, respectively. P. aeruginosa significantly increased in embryos, resulting in abnormal limb development, whereas P. aeruginosa defective in elastase activity partly impaired proliferation. In addition, P. aeruginosa-infected chick embryos significantly stimulated the production of matrix metalloproteinases. Several analyses showed that elevated proteases suppressed the proliferation and survival of chondrogenic cells. The results show that this infection model was a useful assay to determine the virulence mechanism of P. aeruginosa in human after intake of microbiologically contaminated foods.

• Proceedings of the PSK Conference
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• 2002.10a
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• pp.366.1-366.1
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• 2002

Emerging medical technologies for effective and lasting repair of articular cartilage include delivery of cells or cell-seeded scaffolds to a defective site to initiate de novo tissue regeneration. In this respect. the availability of an appropriate biomaterial scaffold is crucial to allow chondrocyte growth and cartilaginous matrix deposition in a three-dimensional geometry. Hyaluronic acid (HA) molecules are anchored to the chondrocyte membrane via receptors, such as CD44. (omitted)

• Journal of Korean Society for Quality Management
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• v.50 no.3
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• pp.425-440
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• 2022

Purpose: The purpose of this paper is to improve the chromaticity coordinate quality of white LED packages for automobiles that require high quality and reliability. Methods: The project follows the structured methodology of the Six Sigma DMAIC Roadmap, which consists of Define, Measure, Analyze, Improve and Control phases. Results: A CTQ is determined based on COPQ analysis, and a process map and a XY matrix are utilized for selecting process input variables. Three vital Few Xs are identified through data analysis; amount to mix at one time, deviation by head pumps, and deviation by production magazines, and process improvements are performed for each of the three vital Few Xs. Conclusion: The improved process conditions for the three vital Few Xs are applied to the production line, and the results show that the percent defective of chromaticity coordinate has improved from 1.59% to 0.63% and a financial effect of about 50 million won per year is obtained.

• The Journal of Korean Academy of Prosthodontics
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• v.24 no.1
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• pp.125-138
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• 1986

It is considered that etching solution or material itself of phosphoric ester cement will induce not a little pulpal irritation, if applied directly onto unsealed dentinal tubules. This study was designed to confirm above consideration by comparing two different conditions between $Ca(OH)_2$-based and non-$Ca(OH)_2$-based group. Posterior teeth of 15 male dogs were selected for this experiment. One experimental group was filled with cement after $Ca(OH)_2$-basing and enamel-etching, the other experimental group after enamel etching without $Ca(OH)_2$-basing. And both of two experimental groups were observed at 2 hours, 15 hours, 3 days, 1 week, 2 weeks, 4 weeks, and 6 weeks after filling. The findings reached to the following conclusions histologically. 1. In both groups, the damaged odontoblasts were atrophied and eventually disappeared. 2. In non-based group at early stage, odontoblasts were severely atrophied and defective areas were appeared between odontoblast cell layers. However, in based group, the odontoblasts were arranged slight irregularly. 3. In non-based group, a small number of undifferentiated cells below the odontoblast cell layers started to appear at 1 week after filling. However, in based group, the undifferentiated cells were appeared at 15 hours after filling. 4. In non-based group, formation of reparative dentin was not begun until late stage of experiment. However, in based group, formation of reparative dentin matrix was begun at 2 weeks after filling and very thickened reparative dentin was formed at 6 weeks after filling. 5. In odontoblast cell layers of both groups, dilated capillaries were observed. 6. Argyrophilic fibers were reticularly condensed in zone of Weil, and they were connected to the pulp tissue and dentin.

• Biomolecules & Therapeutics
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• v.27 no.6
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• pp.530-539
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• 2019

Brain aging is an inevitable process characterized by structural and functional changes and is a major risk factor for neurodegenerative diseases. Most brain aging studies are focused on neurons and less on astrocytes which are the most abundant cells in the brain known to be in charge of various functions including the maintenance of brain physical formation, ion homeostasis, and secretion of various extracellular matrix proteins. Altered mitochondrial dynamics, defective mitophagy or mitochondrial damages are causative factors of mitochondrial dysfunction, which is linked to age-related disorders. Etoposide is an anti-cancer reagent which can induce DNA stress and cellular senescence of cancer cell lines. In this study, we investigated whether etoposide induces senescence and functional alterations in cultured rat astrocytes. Senescence-associated ${\beta}$-galactosidase (SA-${\beta}$-gal) activity was used as a cellular senescence marker. The results indicated that etoposide-treated astrocytes showed cellular senescence phenotypes including increased SA-${\beta}$-gal-positive cells number, increased nuclear size and increased senescence-associated secretory phenotypes (SASP) such as IL-6. We also observed a decreased expression of cell cycle markers, including PhosphoHistone H3/Histone H3 and CDK2, and dysregulation of cellular functions based on wound-healing, neuronal protection, and phagocytosis assays. Finally, mitochondrial dysfunction was noted through the determination of mitochondrial membrane potential using tetramethylrhodamine methyl ester (TMRM) and the measurement of mitochondrial oxygen consumption rate (OCR). These data suggest that etoposide can induce cellular senescence and mitochondrial dysfunction in astrocytes which may have implications in brain aging and neurodegenerative conditions.

• Journal of the Korean Arthroscopy Society
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• v.15 no.2
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• pp.83-91
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• 2011

Purpose: Microfracture has been used as a first-line treatment to repair articular cartilage defects. In this study, a new technique using an extracelluar matrix biomembrane to cover the cartilage lesions after microfracture was evaluated in terms of cartilage repairability and clinical outcome compared with conventional microfracture technique in a prospective randomized trial. Materials and Methods: A total of 53 patients (59 cases) without osteoarthritis who had focal full thickness articular cartilage lesions were randomly assigned in two group. Seventeen patients (17 cases) underwent conventional microfracture procedure (control group) and thirty-six patients (42 cases) received microfracture and placing biomembrane cover (ArtiFilm$^{TM}$) concomitantly (experimental group). Clinical assessment was done through 6 months postoperatively using the subjective International Knee Documentation Committee IKDC questionnaire, and visual analog scale (VAS) for pain and satisfaction. Magnetic resonance imaging (MRI) was performed at 6 months after the operation in all patients. Results: In clinical outcomes, the significant difference was observed between both groups in IKDC, but not in VAS for pain and for satisfaction (final outcomes of IKDC, p=0.001; VAS for pain, p=0.074; VAS for satisfaction, p=0.194). The MRI showed good to complete defect fill (67 to 100%) in 33 patients (78.6%) of experimental group and 4 patients (23.5%) of control group, respectively. In control group, 9 of 17 patients (52.9%) showed poor defect fill (less than 33%), whereas 5 (11.9%) in experimental group (p=0.001). Assessment of peripheral integration revealed no gap formation in 35 patients (83.3%) in experimental group and 6 patients (35.3%) in control group (p=0.001). No serious complications or adverse effects related to the biomembrane were found. Conclusion: Good short-term follow-up clinical results were obtained in the group whose cartilage defects in the knee joint were covered with biomembrane after the microfracture, with the MRI findings confirming the excellent regeneration of the defective cartilage area. This suggests that the surgery to cover the defective area with biomembrane (ArtiFilm$^{TM}$) after the microfracture procedure is a safe, more effective treatment to induce cartilage regeneration.

• Journal of Chest Surgery
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• v.44 no.6
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• pp.406-412
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• 2011

Background: Development of thoracic aortic aneurysms and aortic dissections (TAAD) is attributed to unbearable wall tension superimposed on defective aortic wall integrity and impaired aortic repair mechanisms. Central to this repair mechanisms are well-balanced and adequately functional cellular components of the aortic wall, including endothelial cells, smooth muscle cells (SMCs), inflammatory cells, and adventitial fibroblasts. Adventitial fibroblasts naturally produce aortic extracellular matrix (ECM), and, when aortic wall is injured, they can be transformed into SMCs, which in turn are involved in aortic remodeling. We postulated the hypothesis that adventitial fibroblasts in patients with TAAD may have defects in ECM production and SMC transformation. Materials and Methods: Adventitial fibroblasts were procured from the adventitial layer of fresh aortic tissues of patients with TAAD (Group I) and of multi-organ donors (Group II), and 4-passage cell culture was performed prior to the experiment. To assess ECM production, cells were treated with TNF-${\alpha}$ (50 pM) and the expression of MMP-2/MMP-3 was analyzed using western blot technique. To assess SMC transformation capacity, cells were treated with TGF-${\beta}1$ and expression of SM ${\alpha}$-actin, SM-MHC, Ki-67 and SM calponin was evaluated using western blot technique. Fibroblasts were then treated with TGF-${\beta}1$ (10 pM) for up to 10 days with TGF-${\beta}1$ supplementation every 2 days, and the proportion of transformed SMC in the cell line was measured using immunofluorescence assay for fibroblast surface antigen every 2 days. Results: MMP-3 expression was significantly lower in group I than in group II. TGF-${\beta}1$-stimulated adventitial fibroblasts in group I expressed less SM ${\alpha}$-actin, SM-MHC, and Ki-67 than in group II. SM-calponin expression was not different between the two groups. Presence of fibroblast was observed on immunofluorescence assay after more than 6 days of TGF-${\beta}1$ treatment in group I, while most fibroblasts were transformed to SMC within 4 days in group II. Conclusion: ECM production and SMC transformation are compromised in adventitial fibroblasts from patients with TAAD. This result suggests that functional restoration of adventitial fibroblasts could well be a novel approach for the prevention and treatment of TAAD.

• Molecules and Cells
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• v.43 no.1
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• pp.66-75
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• 2020

Saturated fatty acids contribute to β-cell dysfunction in the onset of type 2 diabetes mellitus. Cellular responses to lipotoxicity include oxidative stress, endoplasmic reticulum (ER) stress, and blockage of autophagy. Palmitate induces ER Ca²⁺ depletion followed by notable store-operated Ca²⁺ entry. Subsequent elevation of cytosolic Ca²⁺ can activate undesirable signaling pathways culminating in cell death. Mitochondrial Ca²⁺ uniporter (MCU) is the major route for Ca²⁺ uptake into the matrix and couples metabolism with insulin secretion. However, it has been unclear whether mitochondrial Ca²⁺ uptake plays a protective role or contributes to lipotoxicity. Here, we observed palmitate upregulated MCU protein expression in a mouse clonal β-cell, MIN6, under normal glucose, but not high glucose medium. Palmitate elevated baseline cytosolic Ca²⁺ concentration ([Ca²⁺]_i) and reduced depolarization-triggered Ca²⁺ influx likely due to the inactivation of voltage-gated Ca²⁺ channels (VGCCs). Targeted reduction of MCU expression using RNA interference abolished mitochondrial superoxide production but exacerbated palmitate-induced [Ca²⁺]_i overload. Consequently, MCU knockdown aggravated blockage of autophagic degradation. In contrast, co-treatment with verapamil, a VGCC inhibitor, prevented palmitate-induced basal [Ca²⁺]_i elevation and defective [Ca²⁺]_i transients. Extracellular Ca²⁺ chelation as well as VGCC inhibitors effectively rescued autophagy defects and cytotoxicity. These observations suggest enhanced mitochondrial Ca²⁺ uptake via MCU upregulation is a mechanism by which pancreatic β-cells are able to alleviate cytosolic Ca²⁺ overload and its detrimental consequences.