• Title/Summary/Keyword: Gamma-$C_2S$

Search Result 731, Processing Time 0.029 seconds

Chronic Hereditary Tyrosinemia Type I with Novel Mutation in FAH Gene (FAH gene novel mutation을 가진 만성형 Hereditary tyrosinemia 1형)

  • Yang, Sungmin;Choi, Hyo Won;Kang, Yun Koo;Lee, Jin-Sung;Namgoong, Mee Kyung
    • Journal of The Korean Society of Inherited Metabolic disease
    • /
    • v.20 no.2
    • /
    • pp.55-62
    • /
    • 2020
  • A 22-month-old girl who had taken iron supplements due to iron deficiency anemia, presented bloody mucoid stool for one month. She had a bruise at the right periorbital area due to minor trauma and hepatosplenomegaly. Laboratory studies showed anemia, thrombocytopenia, elevated alkaline phosphatase (ALP), hypophosphatemia, decreased haptoglobin, hypocomplementemia, negative direct/indirect Coomb's test, normal vitamin D3 level and high PTHi. Wrist x-ray showed no signs of rickets. The abdominal ultrasound showed only accessory spleen. Tandem mass spectrometry was normal. During follow up, bloody stool regressed after seven days of withdrawal of iron supplement and cow milk, and the total CO2 level had been within 15-20 mEq/L with normal anion gap. NGS (next generation sequencing) panel test for evaluation of renal tubular acidosis showed negative results. After low dose steroid and vitamin D supplements under the impression of hypocomplementemic vasculitis, thrombocytopenia, C3/C4, decreased haptoglobin, and elevated ALP level became normal. At 57 months of age, laboratory findings showed elevated liver enzyme, ALP and gamma-glutamyl transferase again. And liver cirrhosis with splenomegaly and diffuse renal disease were reported with abdomen CT scan. Liver biopsy reported macro- and micronodular cirrhosis. Urine organic acid profile showed elevated succinylacetone level. Whole exome sequencing revealed novel compound heterozygous mutations (NM_00137.2:c.107T>C, NM_00137, 2:c.614T>C) in FAH gene and confirmed by Sanger sequencing. Consequently, the patient was diagnosed as chronic hereditary tyrosinemia type I. She started low phenylalanine/tyrosine diet and nitisinone treatment. Our case had presented symptoms very slowly, which is the first case of chronic tyrosinemia type I in South Korea.

Pheno- and genotyping of Streptococcus iniae isolated from cultured rockfish, Sebastes schlegelii at Korean coastal sites (국내 조피볼락(Sebastes sclegelii) 양식장에서 분리한 Streptococcus iniae의 표현형 및 유전형 특성)

  • Tae-Ho Kim;Hyun-Ja Han;Myoung Sug Kim;Miyoung Cho;Soo-Jin Kim
    • Journal of fish pathology
    • /
    • v.36 no.2
    • /
    • pp.277-286
    • /
    • 2023
  • Korean rockfish, Sebastes schlegelii, is a representative bony fish that belongs to the family Scorpaenidae and the order Scorpaeniformes. It has high ecological and economic value and is widely cultivated in many East Asian countries, including South Korea, Japan and China. One of streptococci, Streptococcus iniae, is Gram-positive cocci with a negative reaction for catalase and oxidase. The Korean rockfish shows clinical signs when infected with S. iniae, such as body darkening, bleeding, enlarged kidneys, blurred eyes, abdominal distension, etc., ultimately leading to death. The Korean rockfish causes significant economic losses every year in South Korea due to streptococcosis. In this study, we identified bacteria from the fish using polymerase chain reaction and conducted analyses of hemolytic activity and biochemical tests using API 20 STREP and API ZYM systems. Results of confirming the hemolytic activity (n=4) observed in alpha-type hemolysis (25%), beta-type hemol- ysis (50%), and gamma-type hemolysis (25%) of isolates. The biochemical test results exhibited sig- nificant variation among S. iniae. Additionally, we performed intraperitoneal injection with S. iniae in the fish and analyzed the phylogenetic tree using housekeeping genes of S. iniae, including cpsD, arcC, glnA, groEL, gyrB, mutS, pheT, prkC, rpoB, and tkt, via multilocus sequence typing (MLST). The lethal dose (LD50) showed strong pathogenicity, such as 3.34 × 10 colony-forming unit (CFU)/ml for 23FBStr0601 strain and 7.16 × 10 CFU/ml for 23FBStr0602 strain. 23FBStr0603 strain showed relatively low pathogenicity at 1.73 × 105 CFU/ml. The strains 23FBStr0601 and 23FBStr0602, which showed strong pathogenicity, clustered into one monophyletic group. The 23FBStr0603 strain showed weak pathogenicity and formed a monophyletic group with KCTC 3657.

Physicochemical Composition of Petasites japonicus S. et Z. Max. (머위 (Petasites japonicus S. et Z. Max.)의 이화학적 성분)

  • Cho, Bae-Sick;Lee, Jae-Joon;Ha, Jin-Ok;Lee, Myung-Yul
    • Food Science and Preservation
    • /
    • v.13 no.5
    • /
    • pp.661-667
    • /
    • 2006
  • This study was carried out to analyze the major chemical component of the leaf and stem of dried Petasites japonicus S. et Z. Max.. Comparing proximate composition of leaf and stem of Petasites japonicus as dry matter basis, stem contained higher moisture, crude fat, crude ash and carbohydrate, with less crude protein. The main component of free sugar and disaccharide in both leaf and stem were fructose and sucrose, respectively. Total amino acids of leaf and stem were 6,853.32 mg% and 2,350.61 mg% respectively. Although the amino acid composition of leaf and stem were different glutamic acid and aspartic acid were the major amino acids in samples. The major fatty acids of total lipids were linolenic acid in leaf and linoleic acid in stem. The ratios of unsaturated fatty acids to saturated fatty acid were 3.93 in leaf and 3.44 in stem. The unsaturated fatty acid content of samples were 3 times higher than those of saturated fatty acid contents. Oxalic acid was the major organic acids in leaf and stem. The contents of vitamin A, C and E were higher in leaf than in stem. The mined compositions of both leaf and stem were composed in order of K, Mg, Ca, Fe, Na, and Zn.

EFFECTS OF POLYPHENOLS OF Cocos Nucifera HUSK FIBRE ON SELECTED KIDNEY FUNCTION INDICES IN MICE

  • Adebayo, Joseph Oluwatope;Owolabi, O.O.;Adewumi, O.S.;Balogun, E.A.;Malomo, S.O.
    • CELLMED
    • /
    • v.9 no.1
    • /
    • pp.2.1-2.6
    • /
    • 2019
  • Decoction of Cocos nucifera husk fibre is used indigenously in Nigeria for malaria treatment. Polyphenols have been identified as the phytochemicals responsible for the antimalarial activity of Cocos nucifera husk fibre, though their toxicity has not been evaluated. The polyphenols of Cocos nucifera husk fibre were therefore evaluated for their effects on selected kidney function indices in mice. Fifty mice were randomly divided into five groups (A-E) of ten mice each. Mice in group A were orally administered 5% DMSO solution while those in groups B, C, D and E were orally administered 31.25, 62.5, 125 and 250 mg/Kg body weight of the polyphenols respectively for seven days. Serum urea, creatinine and uric acid concentrations were determined. Serum levels of sodium, potassium, chloride and calcium ions and kidney alkaline phosphatase (ALP), glutamate dehydrogenase (GDH) and gamma-glutamyltransferase (GGT) activities were also determined. The results showed that the polyphenols significantly reduced (p<0.05) urea concentration at 250 mg/Kg body weight and creatinine concentration at all doses compared to controls. The polyphenols caused no significant alteration (p>0.05) in serum uric acid concentration and kidney ALP, GGT and GDH activities compared to controls. There was significant increase (p<0.05) in serum sodium ion concentration at 31.25, 125 and 250 mg/Kg body weight of polyphenols whereas significant increase (p<0.05) in serum potassium and chloride ions was observed at 62.5 and 250 mg/Kg body weight compared to controls. Thus, polyphenols of Cocos nucifera husk fibre may adversely affect some osmoregulatory functions of the kidney, especially at higher concentrations.

Anti-obesity effect of Polygala tenuifolia (원지(Polygala tenuifolia)의 항비만 효과)

  • Hwang, Ju-Young;Wu, Yong-Xiang;Hwang, Dae-Il;Bae, Suk-Jae;Kim, Taewan
    • Food Science and Preservation
    • /
    • v.21 no.1
    • /
    • pp.97-106
    • /
    • 2014
  • This study was performed in order to investigate the anti-obesity effect of Polygala tenuifolia on lipid mechanism in 3T3-L1 adipocytes. The chemical composition of the P. tenuifolia was analyzed in order to assess its nutritional value. Total dietary fiber was the highest among the proximate component of the P. tenuifolia. These results showed that the P. tenuifolia may be used as a potential functional ingredient for anti-obesity effect. Intracellular lipid droplets in the adipocyte were stained with oil-red O dye and quantified. In comparison to the control, lipid accumulation was significantly decreased by 40.1% and 22.4% when treated with the water extract and 70% EtOH extract of the P. tenuifolia at the concentration of $10{\mu}g/mL$, respectively. The anti-adipogenic effect of the water extract was stronger than that of the 70% EtOH extract. The gene expression levels were measured via Western blot and real-time PCR. As a result, the water extract was found to have decrease the gene expression of SREBP-1c, PPAR, $C/EBP{\alpha}$, FAS, ACC in a dose-dependent manner. These indicate that the water extract inhibits pre-adipocyte differentiation and adipogenesis by blocking the SREBP-1c gene expression in 3T3-L1 cells. Therefore, P. tenuifolia can be used as an effective anti-obesity agent.

Sputtering Yield and Secondary Electron Emission Coefficient(${\gamma}$) of the MgO, $MgAl_2O_4$ and $MgAl_2O_4/MgO$ Thin Film Grown on the Cu Substrate by Using the Focused Ion Beam (Cu 기판위에 성장한 MgO, $MgAl_2O_4$$MgAl_2O_4/MgO$ 박막의 집속이온빔을 이용한 스퍼터링수율 측정과 이차전자방출계수 측정)

  • Jung K.W.;Lee H.J.;Jung W.H.;Oh H.J.;Park C.W.;Choi E.H.;Seo Y.H.;Kang S.O.
    • Journal of the Korean Vacuum Society
    • /
    • v.15 no.4
    • /
    • pp.395-403
    • /
    • 2006
  • It is known that $MgAl_2O_4$ has higher resistance to moisture than MgO, in humid ambient MgO is chemically unstable. It reacts very easily with moisture in the air. In this study, the characteristic of $MgAl_2O_4$ and $MgAl_2O_4/MgO$ layers as dielectric protection layers for AC- PDP (Plasma Display Panel) have been investigated and analysed in comparison for conventional MgO layers. MgO and $MgAl_2O_4$ films both with a thickness of $1000\AA$ and $MgAl_2O_4/MgO$ film with a thickness of $200/800\AA$ were grown on the Cu substrates using the electron beam evaporation. $1000\AA$ thick aluminium layers were deposited on the protective layers in order to avoid the charging effect of $Ga^+$ ion beam while the focused ion beam(FIB) is being used. We obtained sputtering yieds for the MgO, $MgAl_2O_4$ and $MgAl_2O_4/MgO$ films using the FIB system. $MgAl_2O_4/MgO$ protective layers have been found th show $24{\sim}30%$ lower sputtering yield values from 0.244 up to 0.357 than MgO layers with the values from 0.364 up to 0.449 for irradiated $Ga^+$ ion beam with energies ranged from 10 kV to 14 kV. And $MgAl_2O_4$ layers have been found to show lowest sputtering yield values from 0.88 up to 0.109. Secondary electron emission coefficient(g) using the ${\gamma}$- FIB. $MgAl_2O_4/MgO$ and MgO have been found to have similar g values from 0.09 up to 0.12 for indicated $Ne^+$ ion with energies ranged from 50 V to 200 V. Observed images for the surfaces of MgO and $MgAl_2O_4/MgO$ protective layers, after discharge degradation process for 72 hours by SEM and AFM. It is found that $MgAl_2O_4/MgO$ protective layer has superior hardness and degradation resistance properties to MgO protective layer.

INFLUENCES OF SILANE CONCENTRATION FOR FILLER SILANIZATION ON THE PROPERTIES OF COMPOSITES (필러의 실란처리농도가 복합레진의 특성에 미치는 영향)

  • 조태희;박상진
    • Restorative Dentistry and Endodontics
    • /
    • v.26 no.1
    • /
    • pp.23-31
    • /
    • 2001
  • The purpose of this study was to search the optimal silane concentrations for filler- silanization of seven experimental composites. Silica filer was a 25micron crushed type. 0.0%, 0.5%, 1.0%, 1.5%, 2.0%, 2.5%, and 3.0% silane($\gamma$-methacrylooxypropyltrimethoxysilane)were added into silica-filler with weight percentage (wt%). Mixtures(silica filler/silane)were reacted at 6$0^{\circ}C$ for 72hours, and crushed into fine particles those were used as fillers for 7 experimental composites. Monomer was a 3 : 1 mixture of Bis-GMA and TEGDMA containing 0.2% tertiary amine and 0.4% camphoroquinone for light curability. A ratio for mixing the monomer and filler was 75% and 25% respectively. Seven experimental composites was classified with the concentration of silane treated, and the specimen number for each test was 10. Specimens with 6mm diameter and 3mm height dimension for measuring the diametral tensile strength were destroyed with 1mm/min cross-head speed on Instron universal testing machine (No. 4467, USA). Shear bond strength was measured on the specimens bonded to bovine enamel etched with 37% phosphoric acid solution for 1 minute Fractured surfaces were observed by SEM (Hitachi S-3200, Japan) among that of the highest values measured from each groups. Following results were obtained: 1. Experimental composites containing silanized filter showed the significantly higher diametral tensile strength and shear bond strength than the composites containing un-silanized fillers(Group1) (p<0.05). 2. In silanized filler composite resins(Group 2~7), Diametral tensile strength of Group 3 showed the significantly higher than that of Group 2 and Group 6(p<0.05). 3. Shear bond strength was higher in Group 3 than that of Group 7 (p<0.05)in silanized fillers composite resins. 4. Fracture surface was formed in resin matrixes on the specimens from composites containing the fillers treated with 0.5% 1.0%, and 1.5% silane. These results mean that the optimal silane concentrations are exist for each fillet with its size and surface area, and that 1.0% is a optimal value for concentration to coat the 25$\mu\textrm{m}$ filler with silane.

  • PDF

Induction of Apoptosis and Inhibition of NO Production by Piceatannol in Human Lung Cancer A549 Cells (A549 인체 폐암세포에서 piceatannol에 의한 apoptosis 유발과 NO 생성의 억제)

  • Choi, Yung-Hyun
    • Journal of Life Science
    • /
    • v.22 no.6
    • /
    • pp.815-822
    • /
    • 2012
  • Piceatannol (trans-3,4,3',5'-tetrahydroxystilbene), a natural stilbene, is an analogue of resveratrol. Although recent experimental data have revealed the health benefit potency of piceatannol, the molecular mechanisms underlying the anti-cancer activity have not yet been studied in detail. In the present study, the further possible mechanisms by which piceatannol exerts its pro-apoptotic action in cultured human lung cancer A549 cells were investigated. Exposure of A549 cells to piceatannol resulted in growth inhibition and induction of apoptosis. Apoptosis induction of A549 cells by piceatannol showed correlation with proteolytic activation of caspase-3, -8, and -9, and concomitant degradation of activated caspase-3 target proteins such as poly (ADP-ribose) polymerase, phospholipase C-${\gamma}1$, ${\beta}$-catenin, and Inhibitor caspase-activated DNase. The increase in apoptosis by piceatannol treatment was also associated with an increase of pro-apoptotic Bax expression and decrease of anti-apoptotic Bcl-2 and Bcl-xL expression, and caused down-regulation of the inhibitor of apoptosis protein family members and up-regulation of Fas and Fas legend. In addition, piceatannol treatment markedly inhibited the expression of mRNA and proteins of inducible nitric oxide (NO) synthase, and the levels of NO production were progressively down-regulated by piceatannol treatment in a dose-dependent fashion. The results indicate that piceatannol may have therapeutic potential against human gastric cancer cells.

Study on the Wear Resistant Characteristics of Medium Carbon Alloy Steel Plasma-Nitrided (플라즈마 질화처리된 중탄소합금강의 내마모특성에 관한 연구)

  • Cho, H.S.;Roh, Y.S.;Shin, H.K.;Lee, S.Y.
    • Journal of the Korean Society for Heat Treatment
    • /
    • v.5 no.4
    • /
    • pp.215-223
    • /
    • 1992
  • This study has been performed to investigate into some effects of temperature, gas mixing ratio and time on the optical microstructure, hardness and wear characteristics of medium carbon alloy steel treated by plasma nitriding. The results obtained from the experiment are summarized as follows: (1) Optical micrographs of AISI 4140 steel plasma-nitrided by the double stage technique have revealed that the nitrided layer is composed of the compound layer and the diffusion layer. The variation in temperature at the first stage gives effects, on the formation of compound layer and the growth rate is shown to be relatively fast at $460^{\circ}C$. (2) The thickness of compound layer has been found to increase with increasing nitrogen percentage in the gas mixture and the holding time. It is therefore recommended that a shorter holding time and a lower nitrogen percentage are more effective to produce a tougher compound layer and a diffusion layer only. (3) X-ray diffraction analysis for AISI 4140 steel has shown that the compound layer consist of ${\gamma}^{\prime}-Fe_4N$ and ${\alpha}-Fe$ and that tough compound layer diffustion layer only can be obtained by the double stage plasmanitriding process. (4) There is also a tendency that the total hardened layer depth increases with increasing temperature, time and nitrogen percentage in the first stage during the double stage plasma nitriding. (5) The wear resistance of plasma nitrided specimens has been found thobe considerably increased compared to the untreated specimens and the amount of increment has appeared to increase further with increasing nitriding temperature, holding time and notrogen percentage of gas mixture in the first stage treatment.

  • PDF

Effect of Irradiation on the Color, Microbiological Quality, and Sensory Attributes of Frozen Ground Beef, Pork, and Chicken after 6 Months at $-6^{\circ}C$

  • Waje, Catherine;Kim, Mi-Yeung;Nam, Ki-Chang;Jo, Cheo-Run;Kim, Dong-Ho;Lee, Ju-Woon;Kwon, Joong-Ho
    • Food Science and Biotechnology
    • /
    • v.17 no.1
    • /
    • pp.212-215
    • /
    • 2008
  • The effect of gamma irradiation on the Hunter color values, microbiological quality, and sensory attributes of frozen ground beef, pork, and chicken was investigated. Fresh meat samples were purchased from local markets, packed and frozen in polyethylene bags, and irradiated at 5 kGy. The Hunter's L-values (lightness) were not significantly different in all the meat samples after irradiation, but the a-values (redness) were higher in the irradiated beef and pork than the non-irradiated ones. After 6 months of storage at $-6^{\circ}C$, the L-values increased in all the meat samples and the a-value in chicken was lower in the irradiated sample than that of the control. The microbial counts decreased in all the samples right after irradiation, but the coliforms and yeasts & molds increased by 1-2 log cycles after 6 months even under frozen state. The overall acceptability of the meat was not affected by irradiation. Panelists had a higher likeness for the increased redness in irradiated beef. In general, only the color changes in meat as a result of irradiation were found to be species-dependent.