• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.5 no.1
• /
• pp.88-93
• /
• 2005

• 한국생물공학회:학술대회논문집
• /
• 2001.11a
• /
• pp.66-69
• /
• 2001

Previously we developed a CHO cell line (CHO-OTC1-A19) expressing the first two enzymes of urea cycle. This cell line showed higher ammonia removal activity and faster growth rate than the vector controlled CHO cells (CHO-neo-5). The purpose of this study was to develop a cell line with higher ammonia removal activity than the cell line developed previously. To accomplish this, we constructed stable CHO cell lines expressing the first three, the first four, or all five enzymes of urea cycle by the stable transfection method. We finally selected CHO-AL-19 cell line expressing the first three, the first four enzymes of the cycle with higher ammonia activity than CHO-OTC1-A19 and CHO-n대-5 cell lines: 40% and 15% higher than those of CHO-neo-5 and CHO-OTC1-A19 cell lines 72 hour after culture started, respectively. It also showed 44% and 10% higher cell viability than CHO-neo-5 and CHO- OTC1-A19 cell lines at higher cell density. In addition, CHO-AL-19 cells showed 45%-60% and about 20% lower ammonia concentration per cell than those of CHO-neo-% and CHO-OTC1-A19 cell lines, respectively. These results indicate that CHO-AL-19 could be used in the production of human therapeutic proteins with higher efficiency.

• Transactions of the Korean Society of Automotive Engineers
• /
• v.20 no.1
• /
• pp.95-105
• /
• 2012

The selective catalytic reduction (SCR) system is a highly-effective aftertreatment device for NOx reduction of diesel engines. Generally, the ammonia ($NH_3$) was generated from reaction mechanism of SCR in the SCR system using the liquid urea as the reluctant. Therefore, the precise urea dosing control is a very important key for NOx and $NH_3$ slip reduction in the SCR system. This paper investigated NOx and $NH_3$ emission characteristics of urea-SCR dosing system based on model-based control algorithm in order to reduce NOx. In the map-based control algorithm, target amount of urea solution was determined by mass flow rate of exhaust gas obtained from engine rpm, torque and $O_2$ for feed-back control NOx concentration should be measured by NOx sensor. Moreover, this algorithm can not estimate $NH_3$ absorbed on the catalyst. Hence, the urea injection can be too rich or too lean. In this study, the model-based control algorithm was developed and evaluated on the numerical model describing physical and chemical phenomena in SCR system. One channel thermo-fluid model coupled with finely tuned chemical reaction model was applied to this control algorithm. The vehicle test was carried out by using map-based and model-based control algorithms in the NEDC mode in order to evaluate the performance of the model based control algorithm.

• Transactions of the Korean Society of Automotive Engineers
• /
• v.19 no.2
• /
• pp.98-104
• /
• 2011

The introduction of a diesel engine into the passenger car and light duty applications in the United States involves significant technical challenges for the automotive makers. This paper describes the SCR System optimization procedure for such a diesel engine application to meet Tier2 Bin5 emission regulation. A urea SCR system, a representative $NO_x$ reduction after-treatment technique, is applied to a 3.0 liter diesel engine. To achieve the maximum $NO_x$ reduction performance, the exhaust system layout was optimized using series of the computational fluid dynamics and the urea distribution uniformity test. Furthermore a comprehensive simulation model for the key factors influencing $NO_x$ reduction performance was developed and embedded in the Simulink/Matlab environment. This model was then applied to the urea SCR system and played a key role to shorten the time needed for SCR control parameter calibration. The potential of a urea SCR system for reducing diesel $NO_x$ emission is shown for FTP75 and US06 emission standard test cycle.

• Neonatal Medicine
• /
• v.18 no.1
• /
• pp.143-147
• /
• 2011

Argininosuccinic aciduria (ASAuria) is a rare autosomal recessive urea cycle disorder. Neonatal presentation of ASAuria is the most common form. It is characterized by lethargy, feeding intolerance, decreased consciousness, and coma after 24 to 72 hours of birth. We describe a rare case of ASAuria in a female neonate who presented with severe hyperammonemia, a typical characteristic of urea cycle disorders. This patient's diagnosis was confirmed by biochemical analyses, and we found that the patient had a point mutation of the argininosuccinate lyase gene, which was homozygous for a novel 556C>T substitution. We have never seen the neonatal form of ASAuria in Korea. Therefore, this is the first report of neonatal onset ASAuria in Korea.

• YAKHAK HOEJI
• /
• v.51 no.3
• /
• pp.214-218
• /
• 2007

In the previous reports, we developed the CO5 by introducing genes for the first and second urea cycle enzymes, carbamoyl phosphate synthetase I (CPS I) and ornithine transcarbamoylase (OTC) into the IBE cell lines producing erythropoietin (EPO). The CO5 have been found out to have 15-20% higher cell growth rate and produce 2-times more EPO than the parental cell line, IBE. To investigate the role of CPS I in CO5 cell line for the cell growth and amount of EPO, we knock-downed CPS I gene expression via siRNA treatment. Expression level of EPO in cell lysate of CO5 was 3-5 fold higher than that of IBE. After siRNA treatment, the cell growth of CO5 was decreased 8-21% and the EPO productivity in the cell Iysate was significantly decreased. However, these changes of the cell growth and EPO productivity were not observed in IBE. These results indicate that CPS I gene expression is important for the increased cell growth and EPO productivity of CO5 cell line.

• 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.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.2 no.1
• /
• pp.77-79
• /
• 2002

The urea cycle, consisting of a series of six enzymatic reactions, plays key roles to prevent the accumulation of toxic nitrogenous compound and synthesize arginine de novo. Five well characterized diseases have been described, resulting from an enzymatic defect in the biosynthesis of one of the normally expressed enzyme. This presentation will focus on two representative diseases; ornithine transcarbamylase(OTC) deficiency and citrullinemia(argininosuccinate synthetase deficiency). OTC deficiency is one of the most common inborn error of urea cycle, which is inherited in X-linked manner. We identified 17 different mutations in 20 unrelated Korean patients with OTC deficiency; L9X, R26P, R26X, T44I, R92X, G100R, R141Q, G195R, M205T, H214Y, D249G, R277W, F281S, 853 del C, R320X, V323M and 10 bp del at nt. 796-805. These mutations occur at well conserved nucleotide sequences across species or CpG hot spot. The L9X and R26X lead to the disruption of leader sequences, required for directing mitochondrial localization of the OTC precursor. Their phenotypes are severe, and neonatal onset. The G100R, R277W and V323M mutations were uniquely identified in patients with late onset OTC deficiency. The other genotypes are associated with neonatal onset. Out of 20 patients with OTC deficiency, only 6 patients are alive; two were liver transplanted, and normal in growth and development at 2, 4 years after transplantation respectively. Citrullinemia is an autosomal recessive disease, caused by the mutations in the argininosuccinate synthetase(ASS) gene. We identified in 3 major mutations in 11 unrelated Korean patients with citrullinemia; G324S, $IVS6^{-2}$ A to G, and 67 bp ins at nt 1125-1126. Among these, the 67 base pair insertion mutation is novel. The allele frequency of each mutation is; G324S(45%), IVS6-2 A to G(32%), and 67 base pair insertion(14%). All patients are diagnosed at neonatal or infantile age. Interestingly, two patients presented with stroke like episode. Out of 11 patients, 5 patients died. Among 6 patients alive, one patient was successfully liver transplanted.

• Journal of the Korean Society of Clothing and Textiles
• /
• v.25 no.3
• /
• pp.569-576
• /
• 2001

The purpose of this study is to develop multifunctional fibers by sticking cinnamon microcapsules on cotton knit. The prepolymer was made from urea-formaldehyde for usage of wall materials of microcapsules. The parameters for adoptable condition are 5000rpm of agitation speed, 1% of dispersions concentration according to the observation with SEM and particle analyzer. The Antimicrobial activity of cotton knit treated with capsule was increased greatly and maintained on the laundering cycle.

• Proceedings of the PSK Conference
• /
• 2002.10a
• /
• pp.338.1-338.1
• /
• 2002

The efficient Erythropoietin(EPO)-expression system in mammalian cells is required for massive production for therapeutic use. Ammonium ion is a major problem in the production of useful proteins by cultured animal cells and therefore it is of importance to devise a system by which a high productivity of human therapeutic recombinant protein can be maintained or enhanced under low ammonium concentration. (omitted)