• Journal of The Korean Society of Inherited Metabolic disease
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• v.4 no.1
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• pp.23-29
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• 2004

Cystinosis, an autosomal recessively inherited lysosomal storage disease, results from impaired transport of the amino acid cystine out of cellular lysosomes. The consequent accumulation and crystallization of cystine destroys tissues, causing growth retardation, Fanconi syndrome, renal failure, eye problems, and endocrinopathies. The gene for cystinosis, CTNS, was mapped to chromosome 17p13. The diagnosis of cystinosis is made by measuring the leukocyte cystine content and the presence of typical corneal crystals on slit-lamp examination is also diagnostic. Since treatment with cysteamine has proved extremely effective, early diagnosis and treatment are critical aspects. We experienced a typical case of cystinosis in a 12-year-old boy with growth retardation.

• BMB Reports
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• v.56 no.12
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• pp.657-662
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• 2023

Neurodegenerative diseases are characterized by distinct protein aggregates, such as those of α-synuclein and tau. Lysosomal defect is a key contributor to the accumulation and propagation of aberrant protein aggregates in these diseases. The discoveries of common proteinopathies in multiple forms of lysosomal storage diseases (LSDs) and the identification of some LSD genes as susceptible genes for those proteinopathies suggest causative links between LSDs and the proteinopathies. The present study hypothesized that defects in lysosomal genes will differentially affect the propagation of α-synuclein and tau proteins, thereby determining the progression of a specific proteinopathy. We established an imaging-based high-contents screening (HCS) system in Caenorhabditis elegans (C. elegans) model, by which the propagation of α-synuclein or tau is measured by fluorescence intensity. Using this system, we performed RNA interference (RNAi) screening to induce a wide range of lysosomal malfunction through knock down of 79 LSD genes, and to obtain the candidate genes with significant change in protein propagation. While some LSD genes commonly affected both α-synuclein and tau propagation, our study identified the distinct sets of LSD genes that differentially regulate the propagation of either α-synuclein or tau. The specificity and efficacy of these LSD genes were retained in the disease-related phenotypes, such as pharyngeal pumping behavior and life span. This study suggests that distinct lysosomal genes differentially regulate the propagation of α-synuclein and tau, and offer a steppingstone to understanding disease specificity.

• Proceedings of the Korean Society of Postharvest Science and Technology of Agricultural Products Conference
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• 2004.10a
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• pp.67-72
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• 2004

Quality of fresh-cut tomato slices was compared during cold storage under various modified atmosphere packaging conditions. Chilling injury of slices in containers sealed with Film A was higher than with Film B; these films had oxygen transmission rates of 87.4 and 60.0 ml $h^{-1}\;m^{-2}\;atm^{-1}$ at $5^{\circ}C\;and\;99\%$ RH, respectively. While slices in containers with an initial atmospheric composition of air, $4\%\;CO_2+1\;or\;20\%\;O_2,\;8\%\;CO_2+1\;or\;20\%\;O_2,\;or\;12\%\;CO_2+20\%\;O_2$ showed fungal growth, slices in containers with $12\%\;CO_2+1\%\;O_2$ did not. Low ethylene in containers enhanced chilling injury. Modified atmosphere packaging provided good quality tomato slices with a shelf-life of 2 weeks or more at $5^{\circ}C$. Experiments were conducted to compare changes in quality of slices of red tomato (Lycopersicon esculentum Mill. 'Sunbeam') fruit from plants grown using black polyethylene or hairy vetch mulches under various foliar disease management systems including: no fungicide applications (NF), a disease forecasting model (Tom-Cast), and weekly fungicide applications (WF), during storage at $5^{\circ}C$ under a modified atmosphere. Slices were analyzed for firmness, soluble solids content (SSC), titratable acidity (TA), pH, electrolyte leakage, fungi, yeasts, and chilling injury. With both NF and Tom-Cast fungicide treatments, slices from tomato fruit grown with hairy vetch (Vicia villosa Roth) mulch were firmer than those from tomato fruit grown with black polyethylene mulch after 12 days storage. Ethylene production of slices from fruit grown using hairy vetch mulch under Tom-Cast was about 1.5- and 5-fold higher than that of slices from WF and NF fungicide treatments after 12 days, respectively. The percentage of water-soaked areas (chilling injury) for slices from tomato fruit grown using black polyethylene mulch under NF was over 7-fold that of slices from tomato fruit grown using hairy vetch under Tom-Cast. When stored at $20^{\circ}C$, slices from light-red tomato fruit grown with black polyethylene or hairy vetch mulches both showed a rapid increase in electrolyte leakage beginning 6 hours after slicing. However, slices from tomato fruit grown using the hairy vetch mulch tended to have lower electrolyte leakage than those grown with black polyethylene mulch. These results suggest that tomato fruit from plants grown using hairy vetch mulch may be more suitable for fresh-cut slices than those grown using black polyethylene mulch. Also, use of the disease forecasting model Tom-Cast, which can result in lower fungicide application than is currently used commercially, resulted in high quality fruit for fresh-cut processing. Experiments were conducted to determine if ethylene influences chilling injury, as measured by percentage of slices exhibiting water-soaked areas in fresh-cut tomato slices of 'Mountain Pride' and 'Sunbeam' tomato (Lycopersicon esculentum Mill.). Ethylene concentration in containers without ventilation significantly increased during storage at $5^{\circ}C$, whereas little or no accumulation of ethylene occurred in containers with one or six perforations. Chilling injury was greatest for slices in containers with six perforations, compared to slices in containers with one perforation, and was over 13-fold greater than that of slices in control containers with no perforations. An experiment was also performed to investigate the effectiveness of including an ethylene absorbent pad in containers on subsequent ethylene accumulation and chilling injury. While ethylene in the no-pad controls increased continually during storage of both 'Mountain Pride' and 'Sunbeam' tomatoes at $5^{\circ}C$ under modified atmosphere conditions, no increase in accumulation of ethylene was observed in containers containing ethylene absorbent pads throughout storage. The ethylene absorbent pad treatment resulted in a significantly higher percentage of chilling injury compared with the no-pad control. In studies aimed at inhibiting ethylene production using AVG during storage of slices, the concentration of ethylene in control containers (no AVG) remained at elevated levels throughout storage, compared to containers with slices treated with AVG. Chilling injury in slices treated with AVG was 5-fold greater than that of controls. Further, we tested the effect of ethylene pretreatment of slices on subsequent slice shelf-life and quality. In slices treated with ethylene (0, 0.1, 1, or $10\;{mu}L\;L^{-1}$) immediately after slicing, ethylene production in non-treated controls was greater than that of all other ethylene pre-treatments. However, pretreatment of slices 3 days after slicing resulted in a different pattern of ethylene production during storage. Ihe rate of ethylene production by slices treated with 1 L $L^{-1}$ ethylene 3 days after slicing was greater during storage than any of the other ethylene treatments. With slices pre-treated with ethylene, both immediately and 3 days after slicing, the rate of ethylene production tended to show an negative correlation with chilling injury. Chemical name used: 1-aminoethoxyvinylglycine (AVG).

• Research in Plant Disease
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• v.15 no.3
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• pp.230-235
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• 2009

Postharvest skin sooty dapple and decay disease of pear fruit often originates at small stain symptoms that occurred during harvest and handling. Experiments were conducted to characterize the effect of timing of application of disease control materials, and to evaluate sequential postharvest applications of fungicides or fungicides and bio-control agents. Fungicides and bio-control agents were increasingly less effective when the period between harvest and application was prolonged. Thiabendazole (TBZ) applied to fruit without artificial wounding or inoculation effectively reduced skin sooty and decay disease when applied within 3 weeks or 6 weeks in 2 years of study. TBZ, Fludioxonil and pyrimethanil were effective in controlling skin sooty and decay disease at artificial wounds inoculated with Cladosporium tenuissimum up to 14 days after inoculation. Application of TBZ at harvest followed 3 weeks later by application of Fludioxonil was superior to application of TBZ at harvest alone. Two bacterial biocontrol agents reduced skin sooty and decay disease at pear wounds inoculated with C. tenuissimum up to 14 days after inoculation with C. tenuissimum, but were ineffective when applied at 28 days after inoculation. Of possible sequential arrangements of fungicide and bio-control treatments, application of the most effective material promptly after harvest generally resulted in the highest level of disease control.

• Food Science and Preservation
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• v.7 no.3
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• pp.241-244
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• 2000

Grapes(Vitis labruscana B) were fumigated with acetic acid at 20mg/liter to reduce storage decay and packaged with polyethylene film(0.03mm), then stored for 90 days at 2$^{\circ}C$. Modified atmosphere packaging reduced slightly weight loss and soluble solids content during storage. Acetic acid fumigation decreased effectively berry shattering and achieved remarkable control of rotting for storage. Two grape cultivars, 'Campbell Early' and 'Sheridan', fumigated with acetic acid had only 0.7~2.9 berries rot compared with 8.3~27.6 berries rot of cluster for grapes that were not fumigated.

• Journal of Dairy Science and Biotechnology
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• v.32 no.2
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• pp.101-104
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• 2014

Food safety is a global health goal, and food-borne disease are a significant public health threat throughout the world. Dairy products are susceptible to contamination through a wide variety of physical, microbial, and chemical hazards. Risks of microbiological hazards are of immediate and serious concern to human health. Milk was inoculated with Bacillus cereus and stored at 10, 15, 20, and $30^{\circ}C$ for 7 days. We monitored the effect of the temperature on growth rate and variance of toxin production. The growth rate of B. cereus was suppressed in low temperature. We confirmed that the growth rate and the toxin production were accelerated when the storage temperature was increased. B. cereus began to produce toxins when the number of bacteria was higher than $10^7CFU/mL$. Therefore, managing the storage temperature of milk is important to inhibit the growth and the toxin production of B. cereus.

• Journal of Applied Biological Chemistry
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• v.61 no.4
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• pp.405-410
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• 2018

Pseudomonas tolaasii strain No. 6264 has been isolated from mushroom tissue and identified as one of the major pathogen causing brown blotch disease. It secretes peptide toxins, known as tolaasin and its analogue peptides. P. tolaasii 6264 has been used as a typical pathogenic strain to study the brown blotch disease for last 20 years after confirming its blotch-forming ability, hemolytic activity, and white line formation. In this study, the characteristics of P. tolaasii 6264 strain were analyzed and compared according to storage period. Strains of P. tolaasii 6264 stored annually since 2012 were cultured and their pathogenic characters were analyzed. When the 16S rRNA sequences were compared, all strains were divided into two groups. Pathogenic characters including hemolytic activity, blotch-forming ability, and white line test were also investigated. The strains, P. tolaasii 6264-15-2 and P. tolaasii 6264-17, had all three activities; however, the rest of stored strains showed only blotch-forming ability losing other pathogenic characters. Tolaasin peptides were purified from the bacterial cultures and analyzed by mass spectrometry. The strains, P. tolaasii 6264-15-2 and P. tolaasii 6264-17, secreted Tol I (1987 Da), Tol II (1943 Da), and its analogues (1973 Da, 2005 Da) while some of these peptides were not found in the media cultured other strains. These results indicate that the pathogenicity of P. tolaasii could be varied during the storage period.

• Molecules and Cells
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• v.28 no.3
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• pp.139-148
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• 2009

Cell death has been traditionally classified in apoptosis and necrosis. Apoptosis, known as programmed cell death, is an active form of cell death mechanism that is tightly regulated by multiple cellular signaling pathways and requires ATP for its appropriate process. Apoptotic death plays essential roles for successful development and maintenance of normal cellular homeostasis in mammalian. In contrast to apoptosis, necrosis is classically considered as a passive cell death process that occurs rather by accident in disastrous conditions, is not required for energy and eventually induces inflammation. Regardless of different characteristics between apoptosis and necrosis, it has been well defined that both are responsible for a wide range of human diseases. Glycogen storage disease type I (GSD-I) is a kind of human genetic disorders and is caused by the deficiency of a microsomal protein, glucose-6-phosphatase-${\alpha}$ ($G6Pase-{\alpha}$) or glucose-6-phosphate transporter (G6PT) responsible for glucose homeostasis, leading to GSD-Ia or GSD-Ib, respectively. This review summarizes cell deaths in GSD-I and mostly focuses on current knowledge of the neutrophil apoptosis in GSD-Ib based upon ER stress and redox signaling.

• Research in Plant Disease
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• v.23 no.4
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• pp.372-378
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• 2017

In this study, we identified the causative agent of post-harvest gray mold on broccoli that was stored on a farmers' cooperative in Pyeongchang, Gangwon Province, South Korea, in September 2016. The incidence of gray mold on broccoli was 10-30% after 3-5 weeks of storage at $3^{\circ}C$. Symptoms included brownish curd and gray-to-dark mycelia with abundant conidia on the infected broccoli curds. The fungus was isolated from infected fruit and cultured on potato dextrose agar. To identify the fungus, we examined the morphological characteristics and sequenced the rDNA of the fungus and confirmed its pathogenicity according to Koch's postulates. The results of the morphological examination, pathogenicity test, and sequencing of the 5.8S rDNA of the internal transcribed spacer regions (ITS1 and ITS4) and three nuclear protein-coding genes, G3PDH, HSP60, and RPB2, revealed that the causal agent of the post-harvest gray mold on broccoli was Botrytis cinerea. To our knowledge, this is the first report of post-harvest gray mold on broccoli in Korea.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.615-619
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• 2003

Cystinosis, an autosomal recessively inherited lysosomal storage disease, results from impaired transport of the amino acid cystine out of cellular lysosomes. The consequent accumulation and crystallization of cystine destroys tissues, causing growth retardation, Fanconi syndrome, renal failure, eye problems, and endocrinopathies. The gene for cystinosis, CTNS, was mapped to chromosome 17p13. The diagnosis of cystinosis was made by measuring the leukocyte cystine content. The presence of typical corneal crystals on slit-lamp examination is also diagnostic. Since treatment with cysteamine has proved extremely effective, early diagnosis and treatment are critical aspects. We experienced a typical case of cystinosis in a 12-year-old boy with growth retardation.