• Clinical and Experimental Pediatrics
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• v.57 no.9
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• pp.416-419
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• 2014

X-linked adrenoleukodystrophy (X-ALD) is a rare peroxisomal disorder, that is rapidly progressive, neurodegenerative, and recessive, and characteristically primary affects the central nervous system white matter and the adrenal cortex. X-ALD is diagnosed basaed on clinical, radiological, and serological parameters, including elevated plasma levels of very long chain fatty acids (VLCFA), such as C24:0 and C26:0, and high C24:0/C22:0 and C26:0/C22:0 ratios. These tests are complemented with genetic analyses. A 7.5-year-old boy was admitted to Department of Pediatrics, Chungnam National University Hospital with progressive weakness of the bilateral lower extremities. Brain magnetic resonance imaging confirmed clinically suspected ALD. A low dose adrenocorticotropic hormone stimulation test revealed parital adrenal insufficiency. His fasting plasma levels of VLCFA showed that his C24:0/C22:0 and C26:0/C22:0 ratios were significantly elevated to 1.609 (normal, 0-1.390) and 0.075 (normal, 0-0.023), respectively. Genomic DNA was extracted from peripheral whole blood samples collected from the patient and his family. All exons of ABCD1 gene were amplified by polymerase chain reaction (PCR) using specific primers. Amplified PCR products were sequenced using the same primer pairs according to the manufacturer's instructions. We identified a missense mutation (p.Arg163Leu) in the ABCD1 gene of the proband caused by the nucleotide change 488G>T in exon 1. His asymptomatic mother carried the same mutation. We have reported an unpublished mutation in the ABCD1 gene in a patient with X-ALD, who showed increased ratio of C24:0/C22:0 and C26:0/C22:0, despite a normal VLCFA concentrations.

• Asian Journal of Atmospheric Environment
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• v.11 no.1
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• pp.15-28
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• 2017

The adsorptive removal properties of synthetic A4 zeolite were investigated against a total of 16 offensive odors consisting of reduced sulfur compounds (RSCs), nitrogenous compounds (NCs), volatile fatty acids (VFAs), and phenols/indoles (PnI). Removal of these odors was measured using a laboratory-scale impinger-based adsorption setup containing 25 g of the zeolite bed (flow rate of $100mL\;min^{-1}$). The high est and lowest breakthrough (%) values were shown for PnIs and RSCs, respectively, and the maximum and minimum adsorption capacity (${\mu}g\;g^{-1}$) of the zeolite was observed for the RSCs (range of 0.77-3.4) and PnIs (0.06-0.104), respectively. As a result of sorptive removal by zeolite, a reduction in odor strength, measured as odor intensity (OI), was recorded from the minimum of approximately 0.7 OI units (indole [from 2.4 to 1.6]), skatole [2.2 to 1.4], and p-cresol [5.1 to 4.4]) to the maximum of approximately 4 OI units (methanethiol [11.4 to 7.5], n-valeric acid [10.4 to 6.5], i-butyric acid [7.9 to 4.4], and propionic acid [7.2 to 3.7]). Likewise, when removal was examined in terms of odor activity value (OAV), the extent of reduction was significant (i.e., 1000-fold) in the increasing order of amy acetate, i-butyric acid, phenol, propionic acid, and ammonia.

• Toxicological Research
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• v.28 no.3
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• pp.159-164
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• 2012

Allergic skin inflammation such as atopic dermatitis (AD) is characterized by edema and infiltration with various inflammatory cells such as mast cells, basophils, eosinophils and T cells. Thymic stromal lymphopoietin (TSLP) is produced mainly by epidermal keratinocytes, as well as dermal fibroblasts and mast cells in the skin lesions of AD. Omega-3 polyunsaturated fatty acids in fish oil can reduce inflammation in allergic patients. Fermentation has a tremendous capacity to transform chemical structures. The antiinflammatory effects of fish oil have been described in many diseases, but the beneficial effects by which fermented olive flounder oil (FOF) modulates the allergic response is poorly understood. In this study, we produced FOF and tested its ability to suppress the various allergic inflammatory responses. The ability of FOF to modulate the immune system was investigated using a mouse model of AD. The FOF-treated group showed significantly decreased immunoglobulin E (IgE) and histamine in serum. Also, the increased TSLP expression was significantly inhibited in the FOF group; the FOF-treated group was not appreciably different from the hydrocort cream treatment group. In addition, FOF treatment resulted in a smaller spleen size with reduced the thickness and length compared to the induction group. Splenocytes from mice treated with FOF produced significantly less IFN-${\gamma}$, IL-4, T-box transcription factor (T-bet) and GATA binding protein 3 (GATA3) expression compared with the induction group. These results suggest that FOF may be effective in treating the allergic symptoms of AD. 5.

• Biomolecules & Therapeutics
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• v.19 no.1
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• pp.70-76
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• 2011

Bone remodeling is a dynamic process involving a constant balance between osteoclast-induced bone resorption and osteoblast-induced bone formation. Osteoclasts play a crucial homeostatic role in skeletal modeling and remodeling, and destroy bone in many pathological conditions. Previously, we reported that the hexane soluble fraction of Ficus carica inhibited osteoclast differentiation. Poly unsaturated fatty acids, such as ethyl docosahexaenoate (E-DHA), docosahexaenoic acid (DHA), cis-11,14-eicosadienoic acid (EDA) and eicosapentaenoic acid (EPA), were identified from the hexane soluble fraction of Ficus carica. Among them, E-DHA most potently inhibited osteoclastogenesis in RAW264.7 cells. E-DHA reduced the activities of JNK and NF-$\kappa}B$. E-DHA suppressed the expression of c-Fos and nuclear factor of activated T cells c1 (NFATc1). Interestingly, DHA increased the activity of alkaline phosphatase and expression of bone morphogenetic protein 2 (BMP2) more than E-DHA in MC3T3-E1 cells, suggesting that DHA may induce osteoblast differentiation. The data suggests that a combination of E-DHA and DHA has potential use in the treatment of diseases involving abnormal bone lysis, such as osteoporosis, rheumatoid arthritis and periodontal bone erosion.

• Biomolecules & Therapeutics
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• v.20 no.5
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• pp.470-476
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• 2012

Resveratrol, a chemopreventive agent, is rapidly metabolized in the intestine and liver via glucuronidation. Thus, the pharmacokinetics of resveratrol limits its efficacy. To improve efficacy, the activity of resveratrol was investigated in the context of sphingolipid metabolism in human gastric cancer cells. Diverse sphingolipid metabolites, including dihydroceramides (DHCer), were tested for their ability to induce resveratrol cytotoxicity. Exposure to resveratrol ($100{\mu}M$) for 24 hr induced cell death and cell cycle arrest in gastric cancer cells. Exposure to the combination of resveratrol and dimethylsphingosine (DMS) increased cytotoxicity, demonstrating that sphingolipid metabolites intensify resveratrol activity. Specifically, DHCer accumulated in a resveratrol concentration-dependent manner in SNU-1 and HT-29 cells, but not in SNU-668 cells. LC-MS/MS analysis showed that specific DHCer species containing C24:0, C16:0, C24:1, and C22:0 fatty acids chain were increased by up to 30-fold by resveratrol, indicating that resveratrol may partially inhibit DHCer desaturase. Indeed, resveratrol mildly inhibited DHCer desaturase activity compared to the specific inhibitor GT-11 or to retinamide (4-HPR); however, in SNU-1 cells resveratrol alone exhibited a typical cell cycle arrest pattern, which GT-11 did not alter, indicating that inhibition of DHCer desaturase is not essential to the cytotoxicity induced by the combination of resveratrol and sphingolipid metabolites. Resveratrol-induced p53 expression strongly correlated with the enhancement of cytotoxicity observed upon combination of resveratrol with DMS or 4-HPR. Taken together, these results show that DHCer accumulation is a novel lipid biomarker of resveratrol-induced cytotoxicity in human gastric cancer cells.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.4
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• pp.230-237
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• 2015

Purpose: Various gastrointestinal factors may contribute to maladaptive behavior in children with autism spectrum disorders (ASD). To determine the association between maladaptive behavior in children with ASD and gastrointestinal symptoms such as severity, intestinal microbiota, inflammation, enterocyte damage, permeability and absorption of opioid peptides. Methods: This observational cross-sectional study compared children with ASD to healthy controls, aged 2-10 years. Maladaptive behavior was classified using the Approach Withdrawal Problems Composite subtest of the Pervasive Developmental Disorder Behavior Inventory. Dependent variables were gastrointestinal symptom severity index, fecal calprotectin, urinary D-lactate, urinary lactulose/mannitol excretion, urinary intestinal fatty acids binding protein (I-FABP) and urinary opioid peptide excretion. Results: We did not find a significant difference between children with ASD with severe or mild maladaptive behavior and control subjects for gastrointestinal symptoms, fecal calprotectin, urinary D-lactate, and lactulose/mannitol ratio. Urinary opioid peptide excretion was absent in all children. Children with ASD with severe maladaptive behavior showed significantly higher urinary I-FABP levels compared to those with mild maladaptive behavior (p=0.019) and controls (p=0.015). Conclusion: In our series, maladaptive behavior in ASD children was not associated with gastrointestinal symptoms, intestinal inflammation (no difference in calprotectin), microbiota (no difference in urinary D-lactate) and intestinal permeability (no difference in lactulose/manitol ratio). ASD children with severe maladaptive behavior have significantly more enterocyte damage (increased urinary I-FABP) than ASD children with mild maladaptive behavior and normal children.

• Journal of the Korean Society of Food Culture
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• v.13 no.5
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• pp.519-528
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• 1998

This study was conducted to investigate the changes in lipid components of red pepper under various processing conditions such as drying, exposing to lights, extraction and storage conditions. The material used in this study were Chungyang cultivars which contained the largest amount of capsaicinoids and Dabok cultivars which is most popular among the various cultivars produced in Korea. Total lipid content of Chungyang was 15.7% and those of Dabok, Udeungsang and Hongilpum were about 17%. The neutral lipid content in free lipid of red pepper was $78.5{\sim}80.3%$ Phospholipid and glycolipid content in bound lipid were $53.8{\sim}56.9%$ and $35.3{\sim}38.7%$ respectively. The major fatty acids of lipid were linoleic, palmitic and oleic acid. Linoleic acid was presented mainly in pericarp, seed and placenta, whereas the most of palmitic acid was presented in stem. Lipid content of cut and whole red pepper were decreased $24.7{\sim}28%$ and $18.1{\sim}21.5%$ by drying for 48hrs at $65^{\circ}C$ and $95^{\circ}C$. And lipid content was also decreased $3.5{\sim}3.6%$ when the red pepper powder was exposed to 15,000 lux of incandescent light for 30 days at $40^{\circ}C$ and $4{\sim}4.9%$ to fluorescent light. The lipid was extracted the highest content of $50.5{\sim}51.7%$ by acid solution(pH2) and the lowest content by neutral solution, and the higher the salt concentration, the greater the lipid was extracted. During storage at $4^{\circ}C,\;25^{\circ}C,\;40^{\circ}C$, for 30 days the lipid content was not much changed but linoleic acid content was decreased during storage at the same conditions.

• Korean Journal of Food Science and Technology
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• v.17 no.2
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• pp.60-64
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• 1985

To utilize microbial lipases for hydrolysis of milk fat, optimum reaction conditions and characteristics of enzymatic reactions of lipases originated from Rhizopus delemar, Mucor sp., and Candida cylindracea were investigated. Optimum pH and temperature were pH 5.6 and $45^{\circ}C$ for Rhizopus delemar lipase, pH7.5 and $35^{\circ}C$ for Mucor sp. lipase, and pH7.5 and $35^{\circ}C$ for Candida cylindracea lipase. Optimum lipase concentration and optimum substrate concentration were $600{\sim}800\;units/ml$ and 20% milk fat, regardless of their origin. Km values were 6.06% milk fat for Rhizopus delemar lipase, 7.69% for Mucor sp. lipase and 7.99% for Candida cylindracea lipase. Rate of lipid hydrolysis was Rhizopus delemar lipase>Mucor sp. lipase>Candida cylindracea lipase. As the reaction time was extended, liberation of short chain fatty acids was increased. After 8 hours reaction, capric acid content significantly increased with Candida cylindracea lipase, palmitic acid with Mucor sp. lipase and butyric acid with Rhizopus delemar lipase.

• Korean Journal of Food Science and Technology
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• v.13 no.1
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• pp.53-56
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• 1981

Several tests were conducted to study lipoxygenase activity and off-flavor developement in frozen sweet corn. Fresh corn contained about 60% of total lipoxygenase activity in the germ section. When non-blanched frozen sweet corn was stored at $-10^{\circ}F$, it developed off-flavor and most significant changes in the flavor profile of off-flavored sweet corn was $4{\sim}5$ times higher hexanal peaks. The high hexanal peaks observed in the sterilized sweet corn with added lipoxygenase, alone and in combination with other enzymes, suggested the fact that high hexanal peaks in off-flavored sweet corn could be due to an oxidative reaction of lionleic acid (and other unsaturated fatty acids) catalyzed by lipoxygenase. Based on lipoxygenase activity and linoleic acid content in sweet corn, this reaction occur most heavily in the germ section of sweet corn. There was a significant relationship between flavor score of frozen stored corn-on-the-cob and hexanal peak in the germ section of corn-on-the-cob. This result indicated that hexanal peak could be used as an objective index of off-flavor development in frozen sweet corn.

• Korean Journal of Food Science and Technology
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• v.18 no.4
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• pp.313-318
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• 1986

Four model systems of ground cooked meat (GCM), GCM-water (W), GCM-brine (B) and GCM-soybean sauce (S) were prepared and stored at $6^{\circ}C$ for 5 weeks. The oxidative behavior of lipids in the systems and the antioxidative effects of soybean sauce on the lipid oxidation were studied during the period of storage.Tiobarbituric acid values and peroxide values of the systems of GCM, GCM-B and GCM-W were increased significantly with the storage time, however, those values of GCM-S were hardly changed during the time of t u·eeks storage. The decreases in the contents of dienoic/polyenoic fatty acids of neutral or phospholipid fraction in GCM-S system were found to be the lowest among the systems during the 5 weeks storage. And also the least development of rancid flavor evaluated by sensory score during storage were observed in GCM-S system. These results are suggested that soybean sauce in the systems plays an important role as an antioxidative material on the lipid oxidation.