• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.650-654
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• 2008

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

• Food Science of Animal Resources
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• v.29 no.2
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• pp.151-156
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• 2009

The prevalence of food allergies was investigated using questionnaires with 300 subjects whose ages ranged from 19 to 24 years old and the causative food allergens was analyzed using immunological analysis with serum of the subjects who answered that they have/had food allergy. The questionnaire showed that 11.33% of subjects have/had experience of food hypersensitivity, where the main causative foods were fish, beef, chicken, milk, egg, and pork in order. The meat allergy shared 4.65% (2.33% for beef, 1.66% for chicken, 0.66% for pork) in the prevalence of food allergies. The causative beef allergens were investigated with the serum of 6 subjects who have had beef allergy. Western blots were carried out with the serum of P6 subject who showed a positive reaction to beef extract in ELISA. The two specific bands were detected in beef extract on the PVDF membrane, and no band was detected in extracts of pork and chicken. A calculation of the distance of migration by SDS-PAGE enabled the molecular masses of the two bands to be estimated as 67kDa and 31kDa, respectively. The 67kDa was revealed as bovine serum albumin (BSA) which is one of the important beef allergens as reported previously though an analysis of the N-terminal amino acid sequence. However we could not identify the sequence of 31kDa, probably because they comprised several subunits and were modified proteins such as glycoprotein that were unlikely to be easily degraded by the Edman method. The 31kDa band were dyed with the PAS (periodic acid-schiff reagent), suggesting that it might be a glycoprotein. These results suggested that the 31kDa might be considered as a novel potential beef allergen which is not reported previously, although further studies are needed.

• Korean Journal of Agricultural Science
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• v.18 no.1
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• pp.49-73
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• 1991

To elucidate enzymatic properties of $\alpha$-galactosidases (EC3, 2, 1, 22) from germinated soybean and Aspergillus niger changes in the enzyme activities and oligosaccharide contents during germination of soybean were determined and $\alpha$-galactosidases from germinated soybean and wheat bran culture of Aspergillus niger were purified by ammonium sulfate fractionation, ion exchange chromatography and gel filtration. Their chemical and enzymatic properties were investigated and the results obtained were summarized as follows : 1. $\alpha$-Galactosidase activity of soybean was maximized when it was germinated at $25^{\circ}C$ for 120 hours. And raffinose and stachyose in soybean were decomposed completely after 96 hours and 120 hours of germination, respectively. 2. The highest level of $\alpha$-Galactosidase activity was obtained when Aspergillus niger was grown on wheat bran medium at $30^{\circ}C$ for 96 hours. 3. Soybean $\alpha$-galactosidase was purified by 6.6 fold by ammonium slufate fractionation, ion exchange chromatography on DEAE-Cellulose and Sephadex A-50., and gel filtration on Sephadex G-150. Its specific activity was 825 units/mg protein and the yield was 2.5% of the total activity of crude extracts. 4. Aspergillus niger $\alpha$-galactosidase was purified by 23.7 fold. Its specific activity was 1,229 units/mg protein and the yield was 14% of the total activity of wheat bran culture. 5. The purified $\alpha$-galactosidases of soybean and Aspergillus niger were found to be homogeneous by polyacrylamide gel electrophoresis and by HPLC. 6. Chemical properties of the purified $\alpha$-galactosidases were : 1) The soybean $\alpha$-galactosidase was monomeric and its molecular weight was estimated to be 30,000 by SDS-PAGE whereas the Aspergillus niger $\alpha$-galactosidase was a tetrameric glycoprotein which consisted of identical subunits with molecular weight of 28,000 each.

• Journal of the Korean Society of Food Science and Nutrition
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• v.37 no.12
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• pp.1568-1575
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• 2008

This study was performed to determine alleviating effects of green tea (GT) on the N-nitrosodimethylamine (NDMA) toxicity to mucins in the rat lingual salivary glands. Sprague-Dawley rats were divided into five groups: untreated (Control), one week-NDMA administrated (NDMA), one week-GT administrated after NDMA for one week (NDMAGT), one week-NDMA administrated with GT (GTNDMA), and one-week NDMA administrated with GT after GT for one week (GTGTNDMA). The mucin properties were elucidated by periodic acid Schiff (PAS) reaction, alcian blue (AB) pH 2.5, AB pH 2.5-PAS, aldehyde fuchsin (AF) pH 1.7-AB pH 2.5, and high iron diamine (HID)-AB pH 2.5 staining. The lingual von Ebner's glands of NDMA group showed some changes such as contraction and destruction of the serous acini, decrease and disappearance of the cytoplasmic granules, vacuolation of cytoplasm, and the mucigenous duct cells. Also, in the lingual mucous glands, enlarged lumens, fused acini, disappearance of granules, vacuolation of cytoplasm, and mucigenous duct cells were observed. All the GT administrated groups had a tendency to recover. GTGTNDMA group recovered almost up to the state of the control group. The lingual von Ebner's glands of NDMA group showed a decrease of neutral mucin and the lingual mucous glands showed a decrease of both neutral and acidic mucins decreased in comparison with control group, although mucous acini secreting strong sulfomucin decreased while those secreting non-sulfomucin (sialomucin) increased. All the GT administrated groups had a tendency to recover. The degree of recovery in the GTNDMA group was stronger than in NDMAGT group and that of the GTGTNDMA group was almost up to the state of the control group. Plus, lingual von Ebner's glands of GTGTNDMA group contained much more neutral mucin than in the control group. In conclusion, it is suggested that NDMA exhibit the toxicity which affects on the mucin properties and which green tea alleviates.

• Journal of Periodontal and Implant Science
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• v.26 no.4
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• pp.989-1002
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• 1996

The purpose of this study was to determine the effect of a pulsed Nd:YAG laser irradiation on human gingival tissues. The patients, who were planned to be treated by clinical crown lengthening procedure and gingivectomy, were selected. All the patients received oral hygiene instruction, scaling and root planing at preoperation. The crest of gingival tissue on upper and lower anterior teeth was irradiated by a pulsed Nd:YAG laser(El. EN. EN060, Italy) with a fiber optic of 300 m in contact mode for 20 seconds. Gingival tissues were divided into 4 groups according to the laser power of 1.0W(10Hz, 100mJ), 2.0W(20Hz, 100mJ), 3.0W(30Hz, 100mJ) and 4.0W(40Hz, 100mJ). Immediately after the laser irradiation, the specimens were excised, fixed 10% neutral formalin, sectioned $4-6{\mu}m$ thick, stained by Hematoxylin-Eosin and Periodic Acid Schiff stain and observed under light microscope. The removed tissue depth and the coagulated layer depth due to a laser irradiation by a laser irradiation were measured on the microphotographs. The difference of measurements according to the different laser power was statistical1y analyzed by Kruskal Wallis Test with SAS program. The results were as follows : 1. In histologic findings of irradiated gingival tissues; a. In the irradiated gingival specimen with 1.0W laser power, some vesicles were observed in limited superficial layer of gingival epithelium. b. In the irradiated gingival specimen with 2.0W and 3.0W laser power, the epithelium was almost removed except for the traces of viable basal cell remnants at ret peg, and coagulation necrosis related with the thermal effect of laser was noted. c. In the irradiated gingival specimen with 4.0W laser power, complete removal of epithelium, partial removal of underlying connective tissue, and the coagulation necrosis of subjacent gingival tissue were shown. 2. The removed tissue depth was deeper in the irradiated specimens with higher power. There was a statistical significance in the difference of removed tissue depth between 1.0W group ($44.54{\pm}6.99um$) and 3.0W group ($99.75{\pm}6.64{\mu}m$), and between 1.0W group($44.54{\pm}6.99{\mu}m$) and 4.0W group($111.36{\pm}4.50{\mu}m$), and between 2.0W group($98.01{\pm}4.53{\mu}m$) and 4.0W group($111.36{\pm}4.50{\mu}m$)(P<0.05). 3. The coagulated layer depth was deeper in the irradiated specimens with higher power. There was a statistical significance in the difference of coagulated layer depth between 1.0W group($31.82{\pm}8.99{\mu}m$) and 3.0W group($55.99{\pm}20.94{\mu}m$), and between 1.0W group($31.82{\pm}8.99{\mu}m$) and 4.0W group($83.68{\pm}10.34{\mu}m$)(P<0.05). From this study, the results demonstrated that the effects of a pulsed Nd:YAG laser irradiation on gingival tissues seemed to depend on the laser power and that the irradiation with high power could be harmful to adjacent healthy tissue.

• Journal of Life Science
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• v.8 no.5
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• pp.509-519
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• 1998

The effect of N-Nitrosodimethylamine(NDMA) on the glycoconjugates of rat lingual salivary gland was examined by prelectin histochemical methods. Sprague-Dawley rats weighing about 250-300g were divided into control and experimental groups. Each rat of experimental groups was administrated NDMA(17mg/kg) orally and sacrificed in 3, 6, 12, 24, 48, 72, 96 and 120 hours after NDMA administration. The regional differences and change of glycoco-njugates were elucidated by prelectin histochemical methods, such as periodic acid Schiff's(PAS) reaction, alcian blue (AB) pH 2.5, AB pH 0.4, AB pH 2.5-PAS, aldehyde fuchsin(AF) pH 1.7-AB pH 2.5 and high iron diamine(HID)-AB pH 2.5 staining. The major morphological changes in the von Ebner’s gland of NDMA administrated groups were withering and des-truction of serous acini, diminution and disappearance of cytoplasmic granules and vacuolation in cytoplasm of serous cells, and mucinous changes of duct epithelial cells. These changes were noted in NDMA administrated groups for 12 to 72 hours. In the lingual mucous gland of NDMA administrated groups, the major morphological changes were enlargement, fusion and destruction of mucous acini, loss of cytoplasmic granules and vacuolated generation in cytop-lasm of mucous cells, and mucinous change of duct epithelial cells. These changes were severe in NDMA administra-ted groups for 12 to 72 hours. In NDMA administrated groups of lingual von Ebner's gland for 12 and 72 hours, the neutral glycoconjugates be-come diminished remarkably compared to the control group. The decreased amount of neutral glycoconjugates tended to be gradually recovered from 96 hours group. The acidic glycoconjugates which were not detected in control group were found in a few serous cells of these gland of NDMA administrated groups for 6 to 48 hours and 120 ho-urs. The remarkable decrease of neutral and acidic glycoconjugates was observed in the lingual mucous glands 3, 24 and 48 hours after NDMA administration, and the striking decrease of acidic glycoconjugates was found in 72 hours groups. Among acidic glycoconjugates, sulfated glycoconjugates tended to decrease in NDMA administrated groups for 72 hours, while sialic glycoconjugates were increased in NDMA administrated groups for 3, 12 and 48 hours.

• Journal of Life Science
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• v.11 no.6
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• pp.582-594
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• 2001

This experiment was performed to investigate the effects of sulfur dioxide on the histological changes, properties of mucosubstances and glycoconjugates of the nasal respiratory mucosa in the rat. Sprague-Dawley male rats weighing about 200~250g were divided into a control group and SO$_2$ exposed groups. Again SO$_2$ exposed groups were divided into 10 ppm, 25 ppm, 50 ppm, 100 ppm, and 200 ppm subgroups, according to concentrations of SO$_2$ and each SO$_2$exposed groups were divided into 1, 3 and 6 hours groups. For the histological changes, hematoxylin-eosin(H-E) and periodic acid Schiff's(PAS) stainings were used, and for the properties of mucosubstances, PAS, alcian blue (AB) pH 2.5, pH 2.5-PAS, AB pH 1.0 and aldehyde fuchsin (AF) pH1.7-AB pH 2.5 were used. In all the SO$_2$ exposed groups, loss of cilia and detachment of epithelial cells, vacuolation of goblet cells were observed in the respiratory epithelium while epithelial squamous metaplasia and intraepthelial mucous cells were observed in the higher concentration of SO$_2$ and the degree of the loss cilia was higher according as concentration was higher and exposed time was longer. The intraepitheial mucous cells appeared most remarkable in the 50 ppm SO$_2$ exposed group. The numbers of goblet cells and acini of nasal septal gland were varied according to concentration of SO$_2$ and exposed time, but the numbers in the 25 ppm and 50 ppm, SO$_2$ exposed increased remarkably. However, the numbers in the 100 ppm and 200 ppm SO$_2$ exposed group had a tendency to decrease noticeably, or disappeared.

• Tuberculosis and Respiratory Diseases
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• v.54 no.1
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• pp.80-90
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• 2003

Background : Goblet cell hyperplasia is a critical pathological feature in hypersecretory diseases of the airways. A bacterial infection of the lung is also known to induce inflammatory responses, which can lead to the overproduction of mucus. Recently, mucin synthesis in the airways has been reported to be regulated by neutrophilic inflammation-induced epidermal growth factor receptor (EGFR) expression and activation. In addition, it was reported that migration of the activated neutrophils is dependent on the matrix metalloproteinases (MMPs), especially MMP-9. In this study, bacterial lipopolysaccharide (LPS)-induced goblet cell hyperplasia and mucus hypersecretion by EGFR cascade, resulting from the MMPs-dependent neutrophilic inflammation were investigated in the rat airways. Methods : Pathogen-free Sprague-Dawley rats were studied in vivo. Various concentrations of LPS were instilled into the trachea in $300{\mu}{\ell}$ PBS (LPS group). Sterile PBS ($300{\mu}{\ell}$) was instilled into the trachea of the control animals (control group). The airways were examined on different days after instilling LPS. For an examination of the relationship between the LPS-induced goblet cell hyperplasia and MMPs, the animals were pretreated 3 days prior to the LPS instillation and daily thereafter with the matrix metalloproteinase inhibitor (MMPI; 20 mg/Kg/day of CMT-3; Collagenex Pharmaceuticals, USA). The neutrophilic infiltration was quantified as a number in five high power fields (HPF). The alcian blue/periodic acid-Schiff (AB/PAS) stain were performed for the mucus glycoconjugates and the immunohistochemical stains were performed for MUC5AC, EGFR and MMP-9. Their expressions were quantified by an image analysis program and were expressed by the percentage of the total bronchial epithelial area. Results : The instillation of LPS induced AB/PAS and MUC5AC staining in the airway epithelium in a time- and dose-dependent manner. Treatment with the MMPI prevented the LPS-induced goblet cell hyperplasia significantly. The instillation of LPS into the trachea induced also EGFR expression in the airway epithelium. The control airway epithelium contained few leukocytes, but the intratracheal instillation of LPS resulted in a neutrophilic recruitment. A pretreatment with MMPI prevented neutrophilic recruitment, EGFR expression, and goblet cell hyperplasia in the LPS-instilled airway epithelium. Conclusion : Matrix metalloproteinase is involved in LPS-induced mucus hypersecretion, resulting from a neutrophilic inflammation and EGFR cascade. These results suggest a potential therapeutic role of MMPI in the treatment of mucus hypersecretion that were associated with a bacterial infection of the airways.