• Title/Summary/Keyword: $InsP_3$

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A Study on reducing errors in scanning object using a laser s (레이저 스케너를 이용한 측정시 오차감소에 관한 연구)

  • 홍성균;이희관;공영식;양균의
    • Proceedings of the Korean Society of Machine Tool Engineers Conference
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    • 2002.10a
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    • pp.393-398
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    • 2002
  • This study proposes a method to reduce error scanning data of laser scanner. The method co of 3 stages. First, there is an error indu difference of the distance between the prob the object. It is possible to reduce the e planning a scanning strategy: object settin path. Second, the scan data loss of the tooli affects calculating the tooling ball con z-direction compensation is given to calculat accurate registration points. Third, three p used to determine a transformation matrix on frame. As merging, the maximum error usually on the third tooling ball in the conven method, which select a point among three po randomly. We find the centroid of 3 points apply it to determine a new transformation mat

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A Novel Insertion in Exon 23 of the TCOF1 Gene in a Newborn Infant with Treacher Collins Syndrome

  • Yang, Ji Hyeon;Cha, Hyo Hyun;Yoon, Hye Sun
    • Journal of Genetic Medicine
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    • v.10 no.2
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    • pp.109-112
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    • 2013
  • Treacher Collins syndrome (TCS) is the most common and well known mandibulofacial dysostosis with characteristic clinical features including downward slanting of palpebral fissures, coloboma of the lower eyelid, hypoplastic zygomatic arches, micrognathia, macrostomia, microtia, and other deformities of the ears. TCS is caused by mutations in at least 3 genes involved in pre-rRNA transcription: TCOF1, POLR1D and POLR1C. We experienced a 1-day-old female infant with characteristic clinical features of TCS. A novel, heterozygotic mutation within the TCOF1 gene (c.3874_3875insG, p.Ala1292Glyfs*30) was identified to cause a premature stop codon.

AGL gene mutation and clinical features in Korean patients with glycogen storage disease type III

  • Ko, Jung-Min;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of Genetic Medicine
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    • v.4 no.1
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    • pp.72-79
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    • 2007
  • Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

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The Sensitivity Analysis according to Observed Frequency of Daily Composite Insolation based on COMS (관측 빈도에 따른 COMS 기반의 일 평균 일사량 산출의 민감도 분석)

  • Kim, Honghee;Lee, Kyeong-Sang;Seo, Minji;Choi, Sungwon;Sung, Noh-Hun;Lee, Darae;Jin, Donghyun;Kwon, Chaeyoung;Huh, Morang;Han, Kyung-Soo
    • Korean Journal of Remote Sensing
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    • v.32 no.6
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    • pp.733-739
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    • 2016
  • Insolation is an major indicator variable that can serve as an energy source in earth system. It is important to monitor insolation content using remote sensing to evaluate the potential of solar energy. In this study, we performed sensitivity analysis of observed frequency on daily composite insolation over the Korean peninsula. We estimated INS through the channel data of Communication, Ocean and Meteorological Satellite (COMS) and Cloud Mask which have temporal resolution of 1 and 3 hours. We performed Hemispherical Integration by spatial resolution for meaning whole sky. And we performed daily composite insolation. And then we compared the accuracy of estimated COMS insolation data with pyranometer data from 37 points. As a result, there was no great sensitivity in the daily composite INS by observed frequency of satellite that accuracy of the calculated insolation at 1 hour interval was $28.6401W/m^2$ and 3 hours interval was $30.4960W/m^2$. However, there was a great difference in the space distribution of two other INS data by observed frequency of clouds. So, we performed sensitivity analysis with observed frequency of clouds and distinction between the two other INS data. Consequently, there was showed sensitivity up to $19.4392W/m^2$.

The Ornithine Transcarbamylase (OTC) Deficiency Identified by a Novel Mutation (새로운 유전자 돌연변이로 확진된 Ornithine Transcarbamylase (OTC) 결핍증 1례)

  • Song, Ari;Lee, Kiwuk;Yang, Aram;Kim, Jinsup;Park, Hyung-Doo;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.3
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    • pp.148-154
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    • 2016
  • A urea cycle disorder is a condition caused by a defect of the enzymes in the urea cycle, and deficiency of ornithine transcarbamylase (OTC), which converts carbamoyl phosphate and ornithine into citrulline, is the most common type of the disorder. OTC deficiency induces the accumulation of precursors of urea, ammonia, and glutamine, leading to neurological symptoms including hypotonia, respiratory failure, seizure, lethargy, and coma and sometimes to death. Because OTC deficiency is inherited in an X-linked manner, typical symptoms such as vomiting, poor feeding, and lethargy appear mainly in male neonates. We recently had a case that presented with neonatal onset lethargy, vomiting, and apnea in a 4-day-old boy. He was diagnosed with OTC deficiency by biochemical phenotype, including hyperammonemia and an increased orotic acid level in the urine. Genetic analysis of the OTC gene showed a novel mutation c.780_781insCAGGCAGTGT (p.Ile261Glnfs*35). He was treated for hyperammonemia using continuous venovenous hemofiltration (CVVH) at 118 hours after birth. After 4 days of CVVH, his consciousness and blood ammonia concentration were normalized, and he was discharged at the age of 53 days. At around 12 months of age, bilateral femur fractures and osteomyelitis occurred in this patient. Two months after the fracture, he died of septic shock, insulin-resistant hyperglycemia, and multi-organ failure.

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Effect of Steeping Conditions of Corn on Starch Properties (옥수수의 침지조건이 전분의 성질에 미치는 영향)

  • Lee, Eun-Sook;Kim, Sung-Kon
    • Korean Journal of Food Science and Technology
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    • v.22 no.1
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    • pp.99-104
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    • 1990
  • The effects of concentrations of sulfur dioxide (0.16-0.20%) and steeping times (25-50hr) at $52^{\circ}C$ on the changes in pH of steep water and weight and volume of corn and starch properties were investigated. The pH of steep water increased and remained constant after steeping time of 20 hours. The degree of weight or volume gain decreased as the concentration of sulfur dioxide increased. The protein content of starch increased as the concentration of sulfur dioxide increased. Water·binding capacity of starch showed the highest value at the steeping time of 35 hours. Swell ins power and solubility decreased as cocentration of sulfur dioxide and steeping time increased. The initial pasting temperature and peak viscosity were increased and decreased, respectively, as the concentration of sulfur dioxide increased. At the same concentration of sulfur dioxide, the peak viscosity was increased and then decreased. Viscosity of starch in sodium hydroxide solution tended to decrease as the concentration of sulfur dioxide and sleeping time increased. The gel volume of starch in 3M KSCN solution was not affected by concentration of sulfur dioxide and steeping time.

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Studies on Relative Densities of Cockroach Populations in 7 Different Habitats by Using Sticky-Traps in Suwon (끈끈이 트랩(Trap)을 이용한 수원시내 바퀴 개체군의 서식처별 상대밀도 조사)

  • Myung-Soon Kim;Hyo-Sok Yu;Hung-Chul Kim
    • Korean journal of applied entomology
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    • v.34 no.4
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    • pp.391-405
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    • 1995
  • Cockroach survey on the relative density from 7 different habitats including hotel-ins, tea-rooms restaurants, hospitals, apartments, and resident hoses were conducted in 3 District(Ku) areas in Suwon city during the period of February through October, 1994. of a total of 3.039 trap sets, Cockroaches were collected from 1,435 traps, comprised of a 47.22% positive trap-rate. Chinese restaurants were shown to be the highest positive trap-rate of 72.67% of the total while the other habitats such as Korean restaurants, apartments, resident hoses, tea-rooms, hotel-inns, and hospitals were 60.67%, 58.61%, 52.22%, 38.67%, 24.88%, and 17.54%, respectively. Blattella germanica was shown to be the highest population density of 55.01 individuals/trap/week comprised of 97.36% of the total during survey period whereas the other 3 species, Periplaneta japonica, P. americana, and P. fuliginosa constituted in lesser extent of 2.35%, 0.14%, respectively. Of a total of 7 different cockroach breeding habitats, higher population density per trap/week was from restaurants with averages of 20.56 and 8.31 cockroaches from Chinese and Koran restaurants, respectively. An intermediate extent of density was observed from apartments, tea rooms and resident houses with 5.33, 3.79 and 3.53 individuals, respectively. Lower relative densities of cockroaches were observed from hospitals and hotel-inns with averages of 0.18 and 1.00 individuals per trap/week, respectively.

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Changes of Physicochemical Properties of Codonopsis lanceolate Cultivated on a Wild Hill and Stored at Various Conditions (야산 재배더덕의 포장$\cdot$저장 조건에 따른 이화학적 특성 변화)

  • Choi, Moo-Young;Oh, Hae-Sook;Kim, Jun-Ho
    • The Korean Journal of Community Living Science
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    • v.16 no.4
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    • pp.3-13
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    • 2005
  • This study was conducted to investigate the changes in some physicochemical properties of Codonopsis lanceolata cultivated on a wild hill, which were packed with woven polypropylene (WP) or low density polyethylene (LDPE, thickness 0.04 mm) bags and stored at $4^{\circ}C$ or $20^{\circ}C$ for 30 days. Those characteristics of fresh samples were as follows. pH : 5.3, moisture and ash content $86\%$ and $5.5\%$ respectively, Ca, Mg, Na and K content Per Ins dry matter : 427.3mg, 203.4 mg, 10.2mg, 619mg, respectively and crude saponin and codonoposide : 29.7mg and 3.82mg. Among the physicochemical properties, the pH, color parameters and Ca and Mg contents decreased or changed significantly during the storage period, but the content of crude saponin and codonoposide were almost maintained as a fresh sample for 30 days except for the condition of the WP packaging and room temperature. We could conclude that both of the LDPE packaging and chilled conditions were necessary for the best storage of Codonopsis lanceolata cultured on a wild hill for a long time.

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The Effects of Panax ginseng on TCDD-induced Testicular Atrophy in Guinea Pigs

  • Kim, Wun-Jae;Hwang, Seok-Yeon;Lee, Hyung-Lae;Song, Geun-Song;Kim, Si-Kwan
    • Proceedings of the Ginseng society Conference
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    • 1998.06a
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    • pp.300-311
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    • 1998
  • 2,3,7,8-Tetrachlorodibenzo-p-dioxin (TCDD), one of the most notorious toxic environmental pollutants, induces various toxic effects in many organs including testes and is regarded as an endocrine disruptor. Korean ginseng, on the other hand, has been well known for its preventive effects on lox- ins, diabetes melltus and hyperlipidemia. We investigated, histopathologically, the effect of Korean Red ginseng water extract (KR-WE) on guinea pig testes damaged by TCDD. Ninety guinea pigs were divided into 6 groups: normal control (NC) group received vehicle and saline; TCDD,1191kg b.w., was administered intraperitoneally to the single dose TCDD-treated (77) group; 100 mghg b.w.16 and 200mg1kg b.w./d KR-WE were injected intraperitoneally to the preventive groups (PIOO and P2OO, respectively) for 28 days from 1 week before TCDD injection, and to the therapeutic groups (CIOO and C2OO, respectively) for 14 days since 1 week after TCDD administration. Increment of body weight was retarded to a larger extent by TCDD. Moreover, body weight of the 77 group decreased significantly 7 days after TCDD exposure, while that of preventive groups kept increasing. Decrease in body weight was not observed in KR-WE-treated groups. Weight decrease in testes caused by TCDD was remarkably protected by KR-WE. Testicles in 77 group displayed decreased tubular size and maturation arrest at the primary or secondary spermatocyte stage. On the other hand, maturation arrest in germ cells by TCDD was improved in KR-WE treated groups. Almost complete protection of the testes was observed in PIOO and P2OO groups. In addition, the therapeutic effect was noticed in C 100 and C2OO groups. These results provided strong evidence that Korean Red ginseng might be a useful agent for the prevention and treatment of testicular damage induced by environmental pollutants.

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Spontaneous Release of Glycosylphosphatidylinositol (GPI)-anchored Renal Dipeptidase from Porcine Renal Proximal Tubules

  • Park, Sung-Wook;Kang, Bok-Yun;Yoon, Hyun-Joong;Park, Eun-Mi;Choi, Kyong;Lee, Hwang-Hee Blaise;Hooper, Nigel M.;Park, Haeng-Soon
    • Archives of Pharmacal Research
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    • v.25 no.1
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    • pp.80-85
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    • 2002
  • The incubation of porcine renal proximal tubules (PTs) resulted in the release of the Glycosylphosphatidylinositol (GPI)-anchored renal dipeptidase (RDPase, EC 3. 4. 13. 19) from the membrane after a lag period of approximately 6 hours. This spontaneous release of RDPase from the membrane was inhibited by antibiotics. When the incubation supernatant was added back to fresh PTs, both the antibiotic inhibition of RDPase release and the lag period disappeared. The released RDPase reacted with an anti-cross reacting determinant antibody indicating the presence of the Ins (1, 2-cyc)P. These results suggest that bacteria in the PTs, when incubated, grow find Secrete a phosphatidylinmsitol-specific phospholipase C (PIPLC). This enzyme then hydrolyses the GPI-anchored RDPase and is transferable. RDPase was purified following its release from the membrane by this simple and inexpensive method which may also be applied to other GPI-anchored proteins.