• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.16 no.2
• /
• pp.109-114
• /
• 2016

Carbamoyl phosphate synthetase 1 (CPS1) deficiency is an autosomal recessive urea cycle disorder which causes hyperammonemia. CPS1 is the first enzyme step in the urea cycle and almost patients present their symptoms during neonatal period. We report a case of CPS1 deficiency in a boy who developed symptoms including lethargy and seizure at 3 days of age. The ammonia level was up to $2,325{\mu}mol/L$, sodium benzoate (250 mg/kg/d) and high calories of both dextrose and lipid was promptly administered. Central access by experienced pediatric surgeon and emergent continuous hemodialysis by pediatric nephrologist was performed within 3 hours and ammonia was less than $100{\mu}mol/L$ at 5 days of age. Currently, he has showed excellent response to treatments including scavenging drugs and a low-protein diet. Despite of diffuse increasing signal intensity on cerebral white matters and basal ganglia on brain MRI, his development and weight gain were good at the last follow-up at 11 months of age. Molecular assay of the CPS1 gene demonstrated that patient had compound heterozygous for c.1529del ($p.Gly510Alafs^*5$) in exon 14 and c.3142-1G>C (IVS25(-1)G>C) in intron 25 and exon 26 boundary. The splicing mutation was novel mutation and inherited from patient's mother. Here, we report a neonatal lethal type CPS1 deficiency patient having novel mutation.

• Fire Science and Engineering
• /
• v.29 no.6
• /
• pp.1-5
• /
• 2015

In this study, the combustion characteristics of Hinoki Cypress Louver were measured after performing pressure impregnation with aqueous solution of boric acid, borax, and ammonium phosphate. The characteristics measured include ignition time, critical heat flux, and mass loss rate by incident hear flux (25, 30 and $50kW/m^2$). The samples used for the test were $100{\times}100{\times}10mm$, and the 5 min variation for each incident heat flux was measured 3 times. The results show that the ignition time for incident heat flux of $25kW/m^2$ showed a delay effect of 17.4 to 21.3% except for Type C-H. There was no significant difference at 35 and $50kW/m^2$ in the average mass loss rate in Types A-H and D-H, which had lower rates than Type N-H, which was predicted to be higher than that of Type N-H ($10.7kW/m^2$) by 38.22 to 60.46%. It is thus expected that at the time of initial primary fire, there would be a delay effect against fire spread.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• v.34 no.1
• /
• pp.36-48
• /
• 2008

To repair bone defects in the oral and maxillofacial field, bone grafts including autografts, allografts, and artificial bone are used in clinical dentistry despite several disadvantages. The purpose of this study was to evaluate new bone formation and healing in rat calvarial bone defects using hydroxyapatite (HA, $Ca_{10}[PO_4]_6[OH]_2,\;Bongros^{(R)}$, Bio@ Co., KOREA) and tricalcium phosphate (${\beta}-TCP,\;Ca_3[PO_4]_2$, Sigma-Aldrich Co., USA) mixed at various ratios. Additionally, this study evaluated the effects of type I collagen (Rat tail, BD Biosciences Co., Sweden) as a basement membrane organic matrix. A total of twenty, 8-week-old, male Sprague-Dawley rats, weighing 250-300g, were divided equally into a control group (n=2) and nine experimental groups (n=2, each). Bilateral, standardized transosseous circular calvarial defects, 5.0 mm in diameter, were created. In each experimental group, the defect was filled with HA and TCP at a ratio of 100:0, 80:20, 70:30, 60:40, 50:50, 40:60, 30:70, 20:80, and 0:100 with or without type I collagen. Rats were sacrificed 4 and 8 weeks post-operation for radiographic (standardized plain film, Kodak Co., USA), histomorphologic (H&E [Hematoxylin and Eosin], MT [Masson Trichrome]), immunohistochemical staining (for BMP-2, -4, VEGF, and vWF), and elementary analysis (Atomic absorption spectrophotometer, Perkin Elmer AAnalyst $100^{(R)}$). As the HA proportion increased, denser radiopacity was seen in most groups at 4 and 8 weeks. In general radiopacity in type I collagen groups was greater than the non-collagen groups, especially in the 100% HA group at 8 weeks. No new bone formation was seen in calvarial defects in any group at 4 weeks. Bridging bone formation from the defect margin was marked at 8 weeks in most type I collagen groups. Although immunohistochemical findings with BMP-2, -4, and VEGF were not significantly different, marked vWF immunoreactivity was present. vWF staining was especially strong in endothelial cells in newly formed bone margins in the 100:0, 80:20, and 70:30 ratio type I collagen groups at 8 weeks. The calcium compositions from the elementary analysis were not statistically significant. Many types of artificial bone have been used as bone graft materials, but most of them can only be applied as an inorganic material. This study confirmed improved bony regeneration by adding organic type I collagen to inorganic HA and TCP mixtures. Therefore, these new artificial bone graft materials, which are under strict storage and distribution systems, will be suggested to be available to clinical dentistry demands.

• The Journal of Korean Academy of Prosthodontics
• /
• v.38 no.2
• /
• pp.169-177
• /
• 2000

The purpose of this study was to compare the retention of complete cast crown over amalgam ores, composite resin cores, and cast gold cores when cemented with three different luting agents. Eighteen core specimens each of amalgam(Bestaloy, Dong Myung, Seoul, Korea), composite resin (Z100, 3M Dental product, st. Paul, Minn) and type IV gold alloy (Ba-4, Heesung Engelhard Corp., Korea) were made in a customized milling stainless steel die. A wax pattern with a loop attached to occlusal surface was made for each core and a type II gold alloy casting was fabricated. The castings which had clinically acceptable marginal fit were used as test samples. The following luting cements were used to cement cast crowns on each core material : (1) zinc phosphate cement (Confi-dental Products Co., USA) (2) glass-ionomer cement (Fuji Plus, GC Industrial Corp., Tokyo, Japan) (3) resin cement (Panavia 21, Kuraray Co., USA). All cements were mixed according to manufacturers' instructions. A static load of 5kg was then applied for 10 minutes on the crowns. All specimens were stored in saline solution for 24 hours at $37^{\circ}C$ and thermocycled for 500 cycles. After storage and cycling, the tensile bond strengths were measured by using a universal testing machine (Instron Corp., Canton, Mass.) at a crosshead speed of 0.5mm/min. The results were as follows 1. The retentive strength of resin cement was the highest of alt three types of cement for resin core (p<0.05). 2. There was no statistical difference among the retentive strengths of three cements for amalgam core (p>0.05). 3. The retentive strength of resin cement was higher than that of zinc phosphate for cast core, but there was no difference between the retentive strength of glass ionomer cement and those of rein and zinc phosphate cement. 4. The retentive strength of the zinc phosphate cement for amalgam core was the highest of all type of cores.

• Clinical and Experimental Pediatrics
• /
• v.52 no.12
• /
• pp.1383-1387
• /
• 2009

We report a case of an 18-month-old girl with glycogen storage disease type Ib (GSD Ib). Her neutrophil counts had gradually decreased to less than $500/{\mu}L$ by the age of 3 years. However, there were no recurrent bacterial infections. Mutation analysis of the glucose-6-phosphate translocase (G6PT) gene revealed a compound heterozygous missense mutation (Ala148Val/Gly273Asp).

• Restorative Dentistry and Endodontics
• /
• v.16 no.1
• /
• pp.25-40
• /
• 1991

Four root canal sealers, Apatite Root Sealer I and II composed mainly of hydroxyapatite/tricalciumphosphate, Sealapex containing calcium hydroxide, and Roth Sealer composed of zinc oxide - eugenol were compared on the culture of L929 fibroblasts. MIT (Methyl Thiazole Tetrazolium Bromide) colorimetric technique was used to measure the mitochondrial dehydrogenase activity. Results were as follows: 1. Hydroxyapatite/tricalcium phosphate mixed sealers were significantly less toxic compared with calcium hydroxide and zinc oxide - eugenol type sealers. High pH of the calcium hydroxide sealer and release of eugenol component from the zinc oxide - eugenol type sealer were presumed to be the cause of the toxicity of these two sealers. In no cases, there were more cytoblastic effects in hydroxyapatite/tricalcium phosphate mixed sealers compared to the control groups. 2. In all experimental groups, toxicity was decreased as dilutions were increased. However in zinc oxide-eugenol type sealer the cell activity was weakened for all dilution groups. 3. Regarding the effect of setting time, Apatite I and Sealapex were less toxic as the setting progressed. Apatite II kept constant regardless of the different time ellapsed after setting but Roth sealer revealed significantly higher toxicity for all experimental groups. 4. Comparing two different culture periods of 24 hours and 72 hours, Apatite I showed higher cell activities in longer period(72 hours) while Apatite II did not. Sealapex and Roth sealer, however, showed significantly lower cell activities in longer period.

• Molecules and Cells
• /
• v.41 no.4
• /
• pp.351-361
• /
• 2018

Sucrose is a crucial compound for the growth and development of plants, and the regulation of multiple genes depends on the amount of soluble sugars present. Sucrose acts as a signaling molecule that regulates a proton-sucrose symporter, with its sensor being the sucrose transporter. Flavonoid and anthocyanin biosynthesis are regulated by sucrose, and sucrose signaling can affect flavonoid and anthocyanin accumulation. In the present study, we found a Myb transcription factor affecting accumulation of anthocyanin. AtMyb56 showed an increase in its expression in response to sucrose treatment. Under normal conditions, anthocyanin accumulation was similar between Col-0 (wild type) and atmyb56 mutant seedlings; however, under sucrose treatment, the level of anthocyanin accumulation was lower in the atmyb56 mutant plants than in Col-0 plants. Preliminary microarray analysis led to the investigation of the expression of one candidate gene, AtGPT2, in the atmyb56 mutant. The phosphate translocator, which is a plastidial phosphate antiporter family, catalyzes the import of glucose-6-phosphate (G-6-P) into the chloroplast. AtGPT2 gene expression was altered in atmyb56 seedlings in a sucrose-dependent manner in response to circadian cycle. Furthermore, the lack of AtMyb56 resulted in altered accumulation of maltose in a sucrose-dependent manner. Therefore, the sucrose responsive AtMyb56 regulates AtGPT2 gene expression in a sucrose-dependent manner to modulate maltose and anthocyanin accumulations in response to the circadian cycle.

• Journal of Pharmaceutical Investigation
• /
• v.36 no.3
• /
• pp.161-167
• /
• 2006

The objective of this study was to investigate the dissolution patterns of variety of orally administered drug products available on the market. It aimed to understand their dissolution behaviors on the basis of the biopharmaceutics classification system (BCS) concept. On the tenets of BCS, several active pharmaceutical ingredients were selected: fluoxetine hydrochloride (class I), naproxen sodium (class ll), pyridostigmine bromide (class III), furosemide (class IV) and simvastatin (class IV). Typical dissolution media used in this study were pH 1.2, pH 4 & 6.8 phosphate buffers, and water. In cases, particular dissolution media specified in the KP and/or USP were used. Dissolution patterns of fluoxetine hydrochloride and pyridostigmine bromide products were characterized by their rapid release In addition, their dissolution characteristics were relatively unaffected by the type of a dissolution medium. Similar dissolution patterns were observed with pH 1.2, pH 4 & 6.8 phosphate buffers and water. By sharp contrast, poor dissolution patterns were noticed with naproxen sodium products, when pH 1.2 and pH 4 phosphate buffer were used. Improvements in its dissolution were achieved by switching the dissolution media to pH 6.8 phosphate buffer or water. Unsatisfactory dissolution data also were observed with a simvastatin product, when it was subject to dissolution tests by use of a surfactant-free pH 1.2, pH 4 & 6.8 phosphate buffers and water. All the release patterns reported in this study were best understood when BCS concepts were implemented. Our results demonstrated that a BCS-based drug classification should be considered first to choose a dissolution test/method and set up dissolution specification.

• Korean Journal of Environmental Agriculture
• /
• v.26 no.4
• /
• pp.286-296
• /
• 2007

To increase phosphate (P) availability in soils, the efficiency of silicate (Si) in reducing P adsorption was investigated by competitive adsorption tests under changing conditions of pH, ion concentrations, and order of anion addition along with single adsorption properties of each ion at $20^{\circ}C$. In the single ion adsorption study, P and Si ions showed the opposite reaction patterns: phosphate adsorption decreased with increasing pH and attained adsorption maximum however, silicate adsorption increased with increasing pH without attaining adsorption maximum. Phosphorus and Si adsorption were influenced by pH in the range of 5.0 - 9.0 and the type and amount of P and Si concentration. Silicate added to soil before P or in a mixture with P significantly reduced P adsorption above pH 7.0; however, there was no significant Si-induced decreased in P adsorption at pH 5.0 when anions were added as mixture. The efficiency of Si in reducing P adsorption increased with increasing Si concentration and pH. The effect of P on Si adsorption was relatively small at pH 5.0 and no effect of P on silicate adsorption was observed at pH 9.0. The presence of Si strongly depressed P adsorption when Si was added before P compared to P and Si added as a mixture. These results suggest that application of Si may decrease P adsorption and increase the availability of P in soils. Furthermore, a Si source would be better to add before P application to enhance the availability of P in soils.

• Korean Journal of Microbiology
• /
• v.41 no.1
• /
• pp.8-12
• /
• 2005

Some genes, which are involved in the biosynthesis of polysaccharides, could be found by the genome project of Sphingomonas chungbukensis DJ77. In this study, we identified the complete nucleotide sequence of a gene, encoding the glucosyl-isoprenyl phosphate-transferase, which catalyzes the first step in the biochemical pathway for the synthesis of the sphingan type polysaccharide. This gene, named spsB, is initiated by the ATG codon and terminated by the TGA, and its open reading frame consists of 1392 bp, encoding 463 amino acids. The predicted amino acid sequence of this enzyme indicates $50\%$ similarity to SpsB of Sphingomonas spp S88, also produces sphingan, and $48\%$ to GelB of Sphingomonas paucimobilis ATCC 31461.