• Korean journal of applied entomology
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• v.15 no.3 s.28
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• pp.147-151
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• 1976

Laboratory experiments were conducted to evaluate the resistance of the new rice varieties Milyang $\#$21 and $\#$23 at the seedling stage to the brown planthopper(Nilaparvata lugens), small brown planthopper(Laodelphax striatellus), white-back planthopper(Sogatella furcifera), green rorice leafhopper(Nephotettix cincticeps) and zigzag-striped leafhopper (Recilia dorsalis) The varieties Yushin and T(N)-1 were used as susceptible check and the resistant check varieties were Mudgo to brown planthopper, ASD-7 :o small brown planthopper, Colombo to white-back planthopper, IR2061 (46763) to green rice leafhopper and Vellailanalgayan to zigzag-striped leafhopper. The varieties Milyang $\#$21 and $\#$23 were moderate in plant reaction only to the green rice leafhopper and $\#$23 was moderately resistant in plant rection only to the small brown planthopper. The nymphs of plant-and leaf-hoppers were more prefered Milyang $\#$21 and $\#$23 and susceptible check-varieties were more preferred for feeding than the resistant check-varieties. The green rice leafhopper and aigzag·striped leafhopper much more prefered Milyang $\#$21 and $\#$23 for oviposition, while brown planthopper and small brown planthopper more prefered the resistant cheeks than test varieties and susceptible checks. However, there was no any steady relationship in ovipositional preference between resistant and susceptible to the hoppers. Shorter nymphal-periods and higher rate of adult emergence were observed in the test varieties and the susceptible checks compared with the resistant checks. In conclusion, the varieties Milyang $\#$21 and $\#$23 seemed to be lack of true resistance to the plant-and leaf-hoppers from the viewpoints of antibiosis and feeding preference.

• Korean Journal of Agricultural Science
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• v.26 no.1
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• pp.58-64
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• 1999

The chemical composition and ruminal dry matter digestibilities of leaves, stems and roots of Aralia cordata Thunberg were determined and compared each other as a roughage sources for ruminants. The crude protein contents were higher for leaves(12.4%) than for leaves+stems (9.7%), stem(5.1%) and roots (3.8%) (P<0.05). The crude fat contents were higher for leaves (3.7%) than for roots (2.1%) and stems (1.3%) (P<0.05). The crude fiber contents were lower for roots (12.3%) than for leaves (15.0%), leaves+stems (27.7%) and stems (40.3%) (P<0.05), respectively. The contents of neutral detergent fiber were lower for leaves (30.2%) than for leaves+stems (42.0%), roots (50.8%) and stems (60.0%) (P<0.05), respectively. The contents of acid detergent fiber were lower for root(18.3%) than for leaves(21.4%). leaves+stems (37.5%) and stems (49.6%) (P<0.05), respectively. The calcium content of leaves(2.4%) were higher than those of stems and roots (0.97% and 0.69%), however the phosphorus contents were similar among leaves, stems and roots(0.25%, 0.19% and 0.35%). Ruminal dry matter digestibilities for 12, 24, 48 and 72hr of leaves(38.9%, 65.9%, 79.8% and 82.4%) and roots(38.9%, 59.8%, 77.6% and 78.5%) were higher than stems(31.1%, 44.1%, 49.5% and 52.6%). Furthermore the digestibilities of leaves were higher than those of alfalfa hay(37.4%, 48.8%, 67.8% and 71.8%) and although the digestibilities of stems which were the lowest among the parts were higher than those of acasia wood chip(12.6%, 18.2%, 21.6% and 24.3%).

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.31-37
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• 2017

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle that causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed-onset type. Neonatal CPS1D cases often present their symptoms within the first days of life. Delayed-onset cases are predominantly adolescents or adults, and infantile delayed-onset cases are rare. Severe hyperammonemia in the neonatal period leads to serious brain damage, coma, and death if not treated promptly. Therefore, early diagnosis and acute treatment are crucial. Despite the improvement of treatments, including continuous hemodialysis, ammonia-lowering agents, and a low-protein diet, the overall outcome of severe forms of hyperammonemia often remains disappointing. As the liver is the only organ in which ammonia is converted into urea, liver transplantation has been considered as an elegant and radical alternative therapy to classical dietary and medical therapies. However, liver transplantation has many disadvantages, such as a considerable risk for technical complications and perioperative metabolic derangement, especially in neonates. Additionally, there is a lack of suitable donor organs in most countries. According to recent studies, liver cell transplantation is a therapeutic option and serves as a bridge to liver transplantation. Here, we report a Korean CPS1D patient with novel mutations in CPS1 who was treated by liver cell transplantation after being diagnosed in the neonatal period and showed a good neurodevelopmental outcome at the last follow-up at six months of age.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.11-17
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• 2017

Purpose: Phenylketonuria (PKU) results from a deficiency of phenylalanine hydroxylase (PAH). The mutation of the PAH gene results in decreased phenylalanine hydroxylase enzyme activity in hyperphenylalaninemia (HPA) patients. This study reports ten cases of patients with the benign HPA genotype c.158G>A (p.Arg53His, R53H) variant in the PAH gene and aims to evaluate the clinical significance of the R53H variant. Methods: Ten Korean patients with the HPA genotype the R53H variant were included in this study. A retrospective medical record review was conducted. We characterized the phenotypes of the patients with HPA with the R53H variant using the following system: classic PKU, moderate PKU, mild PKU, Mild HPA, and benign HPA. Results: Five patients had the R53H variant with the "Pathogenic" variants (R413P, R241C, $Y356^*$, c.442-1G>A, $Y325^*$), Two patients had the "Likely pathogenic" variants ($W187^*$, A259T), Two patients had the "Uncertain significance" variants (R53H, G344D), and One patient had the "Not provided" variant (c.1066-14C>G). Nine patients genotyped with the R53H variant were the patient with benign HPA and One patient genotyped with the R53H homozygote was within normal range of plasma phenylalanine. None of the ten patients required dietary restriction of phenylalanine or pharmacotherapy to maintain their plasma phenylalanine levels and showed no clinical symptoms of HPA. Conclusion: Ten patients with HPA genotype the R53H variant were the patient with benign HPA and showed no clinical symptoms of HPA. Thus, the R53H variant, which was previously classified as an "Uncertain significance" mutation in HPA patients, should be re-classified as "Benign."

• Korean Journal of Poultry Science
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• v.42 no.1
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• pp.77-86
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• 2015

Chicken is one of the major livestock, especially for supplying proteins to human. The chicken genome size is approximately one-third compared with that of the human genome and regarded as a valuable model animal for genetics and development biology. In this study, we constructed the genetic linkage map for Korean native chicken (KNC) using 131 microsatellite (MS) and 8 single nucleotide polymorphism (SNP) markers. As a result, the total map length was calculated as 2729.4 cM and the average genetic distance between markers was 19.64 cM. The marker orders and genetic distances were well matched with the consensus linkage map except for the physical order of ADL0278 and MCW0351 in GGA8. In addition, the recombination rates in marcrochromosomes were 3.7 times higher than that of microchromosomes. The average numbers of alleles, expected heterozygosity (Hexp) and polymorphic information content (PIC) values were calculated as 5.5, 0.63 and 0.58, respectively. These results will give useful information for the understanding of genetic structure and QTL studies in KNC.

• Korean Journal of Poultry Science
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• v.42 no.1
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• pp.1-8
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• 2015

Recently, duck meat consumption has been rapidly increased because consumers recognized duck meat for healthy food. In relation to this, Korean duck industry need to develop Korean native duck (KND) breed for both conservation perspective and self-sufficient of the breeding stocks. In this study, 24 microsatellite (MS) markers were investigated for classification of KND and commercial duck (CD) breeds in the Korean market. Using these MS markers, the calculated number of alleles (K), expected heterozygosity (He) values and polymorphic information contents (PIC) were 1~16, 0~0.865 and 0~0.841, respectively. Also, the expected probability of identical values in random individuals (PI), random sib ($PI_{sib}$) and random half-sib ($PI_{half-sib}$) were estimated as $1.64{\times}10^{-16}$, $2.60{\times}10^{-7}$ and $1.30{\times}10^{-12}$, respectively. The results indicated that the expected probabilities of identity powers were enough for the individual identification. However, KND and CD breeds were not fully discriminated well using the 24 MS markers, which may CD and KND has shared same origin or crossbred. Therefore, further studies will be ultimately needed for developing a genetically pure line of KND breed even though the DNA markers used. Finally, these results will provide useful information for individual traceability system in ducks.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.93-101
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• 2016

Purpose: McArdle disease, glycogen storage disease type V (GSD V), is one of the most common adolescent-onset glycogen storage diseases. It is caused by recessive mutations in PYGM encoding myophosphorylase, which is critical to glycogen metabolism. Since only a few korean patients have been reported, we will observe the clinical and genetic features of three korean patients with McArdle disease. Methods: We retrospectively reviewed the medical records of three patients with genetically confirmed McArdle disease, including the results of forearm ischemic exercise test, electromyogram, nerve conduction velocity, muscle biopsy, and PYGM analysis in peripheral leukocytes. Results: All three cases were males and their age of symptom onset was 12, 5, 14 years old, respectively. A high basal level of serum creatine kinase was noted in all three patients. They experienced the recurrent episodes of rhabdomyolysis, but second wind phenomenon was not definite. In muscle biopsy, subsarcolemmal space vacuoles including periodic acid schiff stained materials were found in two patients, while no evidence of glycogen storage disease was found in the other. A total of five different mutations, $p.Arg50^*$, p.Trp798Arg, $p.Arg50^*$, p.Glu779del, $p.Asp511Thrfs^*28$ and p.Phe710del, were found in three patients. Avoidance of isometric exercise, aerobic exercise and glucose intake before each exercise were recommended for all patients. Conclusion: The three Korean patients with McArdle disease showed the typical manifestations of the condition. The most mutations were private. Therefore, identification of more cases with long-term follow-up will be required to understand the clinical and genetic features of this disease among Korean population.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.128-136
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• 2003

Purpose : Hyper IgM syndrome(HIGM) is characterized by severe recurrent bacterial infections with decreased serum levels of IgG, IgA, and IgE but elevated IgM levels. Recently, it has been classified into three groups; HIGM1, HIGM2 and a rare form of HIGM. HIGM1 is a X-linked form of HIGM and has now been identified as a T-cell deficiency in which mutations occur in the gene that encodes the CD40 ligand molecule. HIGM2 is an autosomal recessive form of HIGM. Molecular studies have shown that the mutation of HIGM2 is in the gene that encodes activation-induced cytidine deaminase(AID). Recently, another rare form of X-linked HIGM syndrome associated with hypohydrotic ectodermal dysplasia has been identified. We encountered a patient with a varient form of HIGM2. To clarify the cause of this form of HIGM, we evaluated the peripheral B cells of this patient. Methods : The lymphocytes of the patient were prepared from peripheral blood. B cells were immortalized with the infection of EBV. Cell cycle analysis was done with the immortalized B cells of the patient. Peripheral mononuclear cells were stained with monoclonal anti-CD40L antibody. Total RNA was extracted from the peripheral mononuclear cells. After RT-PCR, direct sequencing for CD40L gene and HuAID gene were done. Immunostainings of a lymph node for CD3, CD23, CD40, Fas-L, bcl-2, BAX were done. Results : The peripheral B cells of this patient showed normal expression of CD40L molecule and normal sequencing of CD40L gene, and also normal sequencing of AID gene. Interestingly, the peripheral B cells of this patient showed a decreased population of G2/mitosis phase in cell cycles which recovered to normal with the stimulation of IL-4. Conclusion : We suspect that the cause of increased serum IgM in this patient may be from a decrease of G2/mitosis phase of the peripheral B cells, which may be from the decreased production or secretion of IL-4. Therefore, this may be a new form of HIGM.

• Radiation Oncology Journal
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• v.21 no.3
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• pp.227-237
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• 2003

Purpose: The human chronic myelogenous leukemia cell line, K562, expresses the chimeric bcr-abl oncoprotein, whose deregulated protein tyrosine kinase activity antagonizes via DNA damaging agents. Previous experiments have shown that nanomolar concentrations of herbimycin A (HWA) coupled with X-irradiation have a synergistic effect in inducing apoptosis in the Ph-positive K562 leukemia cell line, but genistein, a PTK inhibitor, is non selective for the radiation-induced apoptosils on $p210^{bcr/abl}$ protected K562 cells. In these experiments, the cytoplasmic signal transduction pathways, the Induction on a number of transcription factors and the differential gene expression in this model were investigated. Materials and Methids: K562 cells in the exponential growth phase were used in this study. The cells were irradiated with 0.5-12 Gy, using a 6 Mev Linac (Clinac 1800, Varian, USA). Immediately after irradiation, the cells were treated with $0.25/muM$ of HMA and $25/muM$ of genistein, and the expressions and the activities of abl kinase, MAPK family, NF- kB, c-fos, c-myc, and thymidine kinase1 (TK1) were examined. The differential gene expressions induced by PTK inhibitors were also investigated. Results: The modulating effects of herbimycin A and genistein on the radiosensitivity of K562 cells were not related to the bcr-abl kinase activity. The signaling responses through the MAPK family of proteins, were not involved either in association with the radiation-induced apoptosis, which is accelerated by HMA, the expression of c-myc was increased. The combined treatment of genistein, with irradiation, enhanced NF- kB activity and the TK1 expression and activity. Conclusion: The effects of HMA and genistein on the radiosensitivity on the K562 cells were not related to the bcr-abl kinase activity in this study, another signaling pathway, besides the WAPK family responses to radiation to K562 cells, was found. Further evaluation using this model will provide valuable information for the optional radiosensitization or radioprotection.

• The korean journal of orthodontics
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• v.33 no.5 s.100
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• pp.359-370
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• 2003

The purpose of this study was to evaluate the bond strength of orthodontic brackets bonded to metal bar with chemically cured adhesive (Ortho-one, Bisco Co, USA) in various types and directions of force application. Three types of metal bracket with different bracket base configurations; Micro-Loc base(Tomy Co, Japan), Chessboard base(Daesung Co, Korea), Non-etched Foil-Mesh base(Dentaurum, Germany); were used in this study. Peel, shear, tensile bond strengths were measured by universal testing machine and compared each other. The peel force directions applied were $0^{\circ},\;15^{\circ},\;30^{\circ},\;45^{\circ},\;60^{\circ},\;75^{\circ},\;90^{\circ}$ And then, in consideration of the different surface area of the bracket bases, the bond strength Per unit area were calculated and compared. The results obtained were summarized as follows: 1. The bond strengths according to the types and the directions of the forces were greatest at the shear forces in all three bracket base configuration groups(p<0.01). 2. As the peel force direction grew higher in degree, peel bond strength decreased. The Patterns of peel bond strength change according to force direction was similar in all three bracket base configurations. The minimum bond strength was 60 degree-peel bond strengths in all three bracket base configurations. 3. In Micro-Loc base group, minimum peel bond strength$(_{60}PBS)$ was in $29\%$ level of shear bond strength and $52\%$ level of tensile bond strength. In Chessboard base group, $_{60}PBS$ was in $34\%$ level of shear bond strength and $61\%$ level of tensile bond strength. In Non-etched Foil-Mesh base group, $_{60}PBS$ was in $34\%$ level of shear bond strength and $55\%$ level of tensile bond strength. 4. The bond strengths per unit area were lowest in Non-etched Foil-Mesh base group and highest in Chessboard base group(p<0.05). However, there were no differences in shear bond strength, tensile bond strength, $75^{\circ}\;and\;90^{\circ}$ per unit area between Micro-Loc and Chessboard base groups.