• Korean Journal of Food Science and Technology
• /
• 제28권6호
• /
• pp.1142-1145
• /
• 1996

Long term-storage of citrus oranges after harvest has been hindered mainly by molds The goal of this research was to collect and identify those molds, which would help find a way to extend shelf-life after harvest. During the period of 1994 to 1995, fourteen different strains were isolated and purified from putrefied fruit (Citrus unshiu var.) that was stored at room temperature under open air. The storage disease was caused by the following molds: Penicillium italicum, 25.8%, Monilia candida, 19.8%; Alternaria citri, 18.1%; Mucor hiemalis, 11.0%; Phomopsis citri, 6.6%; Botrytis cinerea. 5.5%; Phoma citricarpa, 3.8%; Glomerella cingulata, 3.8%; P. digitatum, 1.1%; other molds, 4.5%; Most of the strains showed pectinolytic activity and putrefaction. These citrus fruit-putrefying molds will be used as target strains for the control of microorganisms during post-harvest storage.

• The Plant Pathology Journal
• /
• 제18권4호
• /
• pp.199-203
• /
• 2002

Soft rot occurred severely in onion bulbs stored under low temperature ($5^{\circ}C$) in storage houses at Changyoung, Kyungnam province, Korea in early 2000. Water-soaking and yellowish-brown lesions initially appeared on the outside scales of diseased onion bulbs, gradually progressing into the inside scales. Among the bacterial isolates obtained from the lesions, K-2 isolate was found to be responsible for the disease, which grew at a temperature range of from $0^{\circ}C$ to $36^{\circ}C$ with optimum temperature of $00^{\circ}$-$33^{\circ}C$. However, it showed strong pathogenicity to onion bulbs at $25^{\circ}C$ and $5^{\circ}C$ at 3 days and 2 months, respectively. The bacterium also caused soft rot on potato and showed hypersensitive reactions to tobacco and potato. The causal bacterium of onion soft rot was identified as Pseudomonas marginalis based on morphological, biochemical, and physiological characteristics including LOPAT, Soft rot in onion under low temperature storage caused by P. marginalis has not been previously reported.

• Journal of Korean Society of Environmental Engineers
• /
• 제36권10호
• /
• pp.679-684
• /
• 2014

Many burial sites were constructed to suppress the spread of foot and mouth disease during outbreak. Defected burial sites were removed when leachate leak is presumed and carcasses were moved to the circular storage tanks. However, carcasses were not decomposed possibly due to low water content, low microbial activities, and poor mixing. In this research, storage tank containing carcasses in it was modified to bioreactor to accelerate stabilization. Liquids with nutrients were added and circulated to maintain the optimum water content while extraneous microorganisms were augmented. Settlement was used as the primary index for assessing stabilization rate, and the consolidation theory was utilized to estimate the expected final settlement. 30% of carcasses is expected to be decomposed and removed from the storage tank for five years of bioreactor operation.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제25권4호
• /
• pp.321-331
• /
• 2022

Purpose: This study aimed to assess the quality of life (QoL) of children with glycogen storage disease (GSD) and their parents and to determine the impact of myopathies. Methods: A prospective case-control study was conducted at the Cairo University Children's Hospital and National Liver Institute, Menoufia University. A promising new style of questionnaire called the Stark Quality of Life Questionnaire was used to assess the quality of life. Results: Fifty-two children diagnosed with GSD (cases) and 55 age- and sex-matched healthy children (controls) were included. A statistically significant difference was found between cases and controls regarding food intake; mental behavior parameters such as mood, energy, and social contact; and physical behavior parameters such as running and tying shoelaces. Children with myopathies had significantly lower QoL scores in most of the parameters. Conclusion: GSDs alter children and their parents' mental and physical abilities. Lower QoL scores were detected in children with both skeletal myopathy and cardiomyopathy, but the difference was not statistically significant when compared with the children without myopathies.

• Korean Journal of Fisheries and Aquatic Sciences
• /
• 제55권4호
• /
• pp.425-430
• /
• 2022

This study was designed to verify the shelf-life of a freeze dried-block type of convenience food for rockfish Sebastes schlegeli seaweed soup product stored at different storing temperatures (25, 35, and 45℃) for 5 months. The polyunsaturated:saturated fatty acid ratio of the product stored at 25℃ was higher than that of products stored at 35℃ and 45℃ for 5 months. The colorimetric assessment indicated a noticeable decrease in the brightness of product color after 5months of storage at 35℃ and 45℃. Increased storage temperature and time negatively affected the product color. The products stored at 35℃ and/or 45℃ for more than 3 months tended to be more yellowish-red in color than those stored at 25℃ for shorter periods. No disease-causing microorganisms, including Escherichia coli and Staphylococcus aureus, posing health hazards to the human, were detected on food safety evaluation, regardless of storage conditions. Based on food visual shelf life simulator the shelf life of the rockfish seaweed soup was estimated approximately 22 months, considering the data from yellowness the safety factor of 0.7.

• Journal of Life Science
• /
• 제30권8호
• /
• pp.672-679
• /
• 2020

Distal myopathy is a clinically and genetically heterogeneous group of degenerative diseases of the distal muscle. Glycogen storage disease type IXD (GSD9D) is a metabolic distal myopathy characterized by muscle deficiency of phosphorylase kinase, a key regulatory enzyme in glycogen metabolism. Affected individuals may develop muscle weakness, degeneration, and cramps, as well as abnormal muscle pain and stiffness after exercise. It has been reported that mutations in the PHKA1 gene which encodes the alpha subunit of muscle phosphorylase kinase cause GSD9D. In this study, we examined a Korean GSD9D family with a c.3314T>C (p.I1105T) mutation in the PHKA1 gene. This mutation has not been previously reported in any mutation database nor was it found in 500 healthy controls. The mutation region is well conserved in various other species, and in silico analysis predicts that it is likely to be pathogenic. To date, only seven mutations in the PHKA1 gene have been documented, and this is the first report of Korean GSD9D patients. This study also describes and compares the clinical symptoms and pathological conditions of previously reported cases and these Korean patients. We believe that our findings will be useful for the molecular diagnosis of GSD9D.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• 제17권3호
• /
• pp.96-102
• /
• 2017

Glycogen storage disease type IX (GSD IX) is caused by deficiency of phosphorylase kinase which plays a role in breakdown of glycogen. Mutations in PHKA2 are the most common cause of GSD IX (GSD IXa). Clinical manifestations of GSD IXa include hepatomegaly, elevation of liver enzyme, growth retardation, fasting hypoglycemia, and fasting ketosis. However, the symptoms overlap with those of other types of GSDs. Here, we report Korean familial cases with GSD IXa whose diagnosis was confirmed by targeted exome sequencing. A 4-year old male patient was presented with hepatomegaly and persistently elevated liver enzyme. Liver biopsy revealed swollen hepatocyte filled with glycogen storage, suggesting GSDs. Targeted exome sequencing was performed for the differential molecular diagnosis of various types of GSDs. A hemizygous mutation in PHKA2 were detected by targeted exome sequencing and confirmed by Sanger sequencing: c.3632C>T (p.Thr121Met), which was previously reported. The familial genetic analysis revealed that his mother was heterozygous carrier of c.3632C>T mutation and his 28-month old brother had hemizygous mutation. His brother also had hepatomegaly and elevated liver enzyme. The hypoglycemia was prevented by frequent meals with complex carbohydrate, as well as cornstarch supplements. Their growth and development is in normal range. We suggest that targeted exome sequencing could be a useful diagnostic tool for the genetically heterogeneous and clinically indistinguishable GSDs. A precise molecular diagnosis of GSD can provide appropriate therapy and genetic counseling for the family.

• Journal of Genetic Medicine
• /
• 제4권1호
• /
• pp.72-79
• /
• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• The Plant Pathology Journal
• /
• 제26권1호
• /
• pp.37-48
• /
• 2010

This paper describes a web-based information system for plant disease forecast that was developed for crop growers in Gyeonggi-do, Korea. The system generates hourly or daily warnings at the spatial resolution of $240\;m{\times}240\;m$ based on weather data. The system consists of four components including weather data acquisition system, job process system, data storage system, and web service system. The spatial resolution of disease forecast is high enough to estimate daily or hourly infection risks of individual farms, so that farmers can use the forecast information practically in determining if and when fungicides are to be sprayed to control diseases. Currently, forecasting models for blast, sheath blight, and grain rot of rice, and scab and rust of pear are available for the system. As for the spatial interpolation of weather data, the interpolated temperature and relative humidity showed high accuracy as compared with the observed data at the same locations. However, the spatial interpolation of rainfall and leaf wetness events needs to be improved. For rice blast forecasting, 44.5% of infection warnings based on the observed weather data were correctly estimated when the disease forecast was made based on the interpolated weather data. The low accuracy in disease forecast based on the interpolated weather data was mainly due to the failure in estimating leaf wetness events.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• 제14권1호
• /
• pp.1-9
• /
• 2014

Many inborn errors of metabolism can be completely cured with early detection and early treatment. This is why neonatal screening on inborn errors of metabolism is implemented worldwide. In 1991, the Ministry of Health & Social affairs adopted a nationwide service program for neonatal screening of phenylketonuria, galactosemia, maple syrup urine disease, homocystinuria, histidinemia and congenital hypothyroidism for newborns delivered from low class pregnant women registered in health centers. Government decreased the test items from six to two, PKU and congenital hypothyroidism to increase test numbers with same budget from 1995. 78 laboratories wanted to participate for neonatal screening test in 1999. Government decided to screen six items of PKU, congenital hypothyroidism, maple syrup urine disease, homocystinuria, galactosemia and congenital adrenal hyperplasia from 2006. In 2014, thirteen laboratories are participating. Inter laboratory quality control was started 6 times a year from 1994. In case a patient with an inherited metabolic disease is diagnosed by screening of government program, special milk is provided at government's expense. According to the government project, from 1997 to 2013, 7,080,569 newborns were screened. 144 PKU, 2.451 congenital hypothyroidism were detected. So incidence of PKU is 1/49,170 and congenital hypothyroidism is 1/2,888. The cost benefit of performing screening procedures coupled with treatment has been estimated to be as high as 1.77 times in PKU, 11.11 times in congenital hypothyroidism than cost without screening. By January 2007, many European countries had expanded of their newborn screening programs by inclusion of Tandem mass spectrometry. We are trying to increase the budget to test all newborns for Tandem mass spectrometry from 2016. We are considering four to five central laboratories which cover all newborns and are equipped with tandem mass spectrometer & enzyme immunoassay for TSH, 17OHP & enzyme colorimetric assay for galactose. And I hope to expand test including Wilson disease screening test and lysosomal storage diseases.