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Two Cases of Tyrosinemia; One with Hepatocellular Carcinoma and the other with Acute Liver Failure (타이로신 혈증 2례; 간암이 유발된 1례와 급성 간부전으로부터 회복된 1례의 비교)

  • Kim, Sook Za;Song, Woong Ju;Jeon, Young Mi;Levy, Harvey L.
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.13 no.1
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    • pp.48-53
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    • 2013
  • Tyrosinemia I (fumarylacetoacetate hydrolase deficiency) is an autosomal recessive inborn error of tyrosine metabolism that produces liver failure in infancy or a more chronic course of liver disease with cirrhosis, often complicated by hepatocellular carcinoma in childhood or early adolescence. We studied a 37-year-old woman with tyrosinemia I whose severe liver disease in infancy and rickets during childhood were resolved with dietary therapy. From 14 years of age, she resumed unrestricted diet with the continued presence of the biochemical features of tyrosinemia, yet maintained normal liver function. In adult years, she accumulated only a small amount of succinylacetone. Despite this evolution to a mild biochemical and clinical phenotype, she eventually developed hepatocellular carcinoma. Her fumarylacetoacetate hydrolase genotype consists of a splice mutation, IVS6-1G>T, and a novel missense mutation, p.Q279R. Studies of resected liver revealed the absence of hydrolytic activity and immunological expression of fumarylacetoacetate hydrolase in tumour. In the non-tumoral areas, however, 53% of normal hydrolytic activity and immunologically present fumarylacetoacetate hydrolase were found. This case demonstrates the high risk of liver cancer in tyrosinemia I even in a seemingly favorable biological environment. In this study of tyrosinemia I, Case 2 with negative succinylacetone accumulation and the recovery of acute liver failure was compared with Case 1. Diet restriction and NTBC treatment are crucial to prevent hepatocellular carcinoma until liver transplant can take place and cure the condition. Further studies are needed to examine cases where liver cancer did not result despite clinical symptoms/signs of tyrosinemia type I.

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Composition of Free Amino Acids and Essential Oils in Root of Anthriscus sylvestylis (전조 뿌리의 유리 아미노산과 정유 성분 조성)

  • 김상국;권태용;민기군;이승필;최부술;이상철
    • KOREAN JOURNAL OF CROP SCIENCE
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    • v.41 no.5
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    • pp.521-525
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    • 1996
  • The study was carried out to find compositions of proximate components, free amino acid, and essential oils from root of Anthriscus sylvestylis. Proximate component contents were 7.69% for protein, 1.74% for fat, 2.44% for fiber, and 3.76% for ash. Extract content was 27.68% in fresh root. The compositions of free amino acids consisted 16 kinds. Phenylalanine content was the highest in composition of free amino acids. The essential oils of the root of Anthriscus sylvestylis was examined. $\alpha$-pinene, campreol, ,$\beta$-pinene, sabinene, myrcene, phellandrene, $\alpha$-terpinolene, d-limone, ${\gamma}$-terpinene, p-cymene, $\alpha$-terpinolene, carboxaldehyde, 3-cyc1ohexen-l-carboxaldehyde, 2-nonenal, isobornyl acetate, 4-terpineol, $\beta$-bisabolene, cis-piperitol, p-cymen-8-ol, BHT, methyl eugenol and 2-methoxy-4-vinyl-phenol were identified from the diethylether layers. Recovery yield of essential oils of Anthriscus sylvestylis of root was 0.58%. As a result, it was considered that the plant is worthy of cultivating as spice and medicinal crops.

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The Analysis of Seminal Plasma Proteins by Two-Dimensional Polyacrylamide Gel Electrophoresis (2-DE) in Hanwoo (Korean Native Cattle)

  • Lee, Yong-Seung;Song, Eun-Ji;Yoo, Han-Jun;Park, Joung-Jun;Cheong, Hee-Tae;Yang, Boo-Keun;Park, Choon-Keun
    • Journal of Embryo Transfer
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    • v.25 no.4
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    • pp.281-286
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    • 2010
  • This study was to evaluate the protein profile of seminal plasma using 2-DE in Hanwoo. Seminal plasma was harvested from five mature Hanwoo, and seminal plasma protein was extracted by M-PER Mammalian Protein Extraction Reagent. Proteins were refined by clean-up kit and quantified by Bradford method until total protein was $300\;{\mu}l$. Immobilized pH gradient (IPG) strip was used 18 cm and 3~11 NL. SDS-PAGE was used 12% acrylamide gel. Each gels were visualized by comassie brilliant blue and silver staining. These spots were analyzed by MALDI-TOF MS and searched on NCBInr. The result, 20 proteins of 36 protein spots were searched through peptide sequencing on the NCBInr. 8 proteins profiled by 2-DE were proved through previous bovine studies and the name of each protein was albumin, nucleobindin, clusterin, TIMP-2, spermadhesin Z13, spermadhesin-1 and BSP proteins (BSP 30 kDa and BSP A1/A2). 12 new proteins were ATP synthase, protein MAK16 homolog, Transmembrane protein 214, E3 ubiquitin-protein ligase BRE1A, dual serine/threonine and tyrosine protein kinase, tissue factor pathway inhibitor 2, alpha-actinin-4, RUN domain-containing protein 3B, catenin alpha-1, protein-glutamine gamma-glutamyltransferase 2, plakophilin-1 and inter-alpha-trypsin inhibitor heavy chain H1 has not been previously described in the bovine seminal plasma study. These proteins may be contribute to define the type of proteins affecting fertility of male and improve the fertilizing ability of semen in Hanwoo.

Korean Red Ginseng extract induces angiogenesis through activation of glucocorticoid receptor

  • Sung, Wai-Nam;Kwok, Hoi-Hin;Rhee, Man-Hee;Yue, Patrick Ying-Kit;Wong, Ricky Ngok-Shun
    • Journal of Ginseng Research
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    • v.41 no.4
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    • pp.477-486
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    • 2017
  • Background: Our previous studies have demonstrated that ginsenoside-Rg1 can promote angiogenesis in vitro and in vivo through activation of the glucocorticoid receptor (GR). Furthermore, microRNA (miRNA) expression profiling has shown that Rg1 can modulate the expression of a subset of miRNAs to induce angiogenesis. Moreover, Rb1 was shown to be antiangiogenic through activation of a different pathway. These studies highlight the important functions of miRNAs on ginseng-regulated physiological processes. The aim of this study was to determine the angiogenic properties of Korean Red Ginseng extract (KGE). Methods and Results: Combining in vitro and in vivo data, KGE at $500{\mu}g/mL$ was found to induce angiogenesis. According to the miRNA sequencing, 484 differentially expressed miRNAs were found to be affected by KGE. Among them, angiogenic-related miRNAs; miR-15b, -23a, -214, and -377 were suppressed by KGE. Meanwhile, their corresponding angiogenic proteins were stimulated, including vascular endothelial growth factor, vascular endothelial growth factor receptor-2, endothelial nitric oxide synthase, and MET transmembrane tyrosine kinase. The miRNAs-regulated signaling pathways of KGE were then found by Cignal 45-Pathway Reporter Array, proving that KGE could activate GR. Conclusion: KGE was found capable of inducing angiogenesis both in vivo and in vitro models through activating GR. This study provides a valuable insight into the angiogenic mechanisms depicted by KGE in relation to specific miRNAs.

Analysis of Amino Acids, Vitamins and Minerals of Fruiting Body of Fomitopsis pinicola (소나무 잔나비버섯의 아미노산, 비타민 및 무기물 분석)

  • Ding, J.L.;Shin, H.J.;Cha, W.S.
    • Journal of Life Science
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    • v.16 no.7 s.80
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    • pp.1123-1126
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    • 2006
  • The nutritional composition of Fomitopsis pinicola (F. pinicola) fruiting body has been analyzed for medicinal and edible uses. The contents of crude fibers, carbohydrates, crude protein, moisture, crude fats and ashes were 43.3%, 26.3%, 12.8%, 12.6%, 3.3% and 1.7%, respectively. Eighteen amino acids were found in F. pinicola. Among total amino acids, glutamate content was the highest (457 mg/100 g dry mushroom) and arginine, glycine, valine, aspartate and isoleucine were followed. Concerning free amino acids, glutamine, arginine, trytophan, and glutamate were dominant. The vitamin E content was the highest (276 mg/100 g dry mushroom), then vitamin H and vitamin B, were followed. The mineral contents were as follows: K 165.06 mg, P77.57 mg, Mg 46.11 mg, Fe 21.56 mg, and Ca 16.90 mg based on 100 g dry mushroom.

Anti-Diabetic and Anti-Inflammatory Effects of Green and Red Kohlrabi Cultivars (Brassica oleracea var. gongylodes)

  • Jung, Hyun Ah;Karki, Subash;Ehom, Na-Yeon;Yoon, Mi-Hee;Kim, Eon Ji;Choi, Jae Sue
    • Preventive Nutrition and Food Science
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    • v.19 no.4
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    • pp.281-290
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    • 2014
  • The aim of the present study was to evaluate the anti-diabetic, anti-inflammatory, antioxidant potential, and total phenolic content (TPC) of green and red kohlrabi cultivars. Anti-diabetic and anti-inflammatory activities were evaluated via protein tyrosine phosphatase (PTP1B) and rat lens aldose reductase inhibitory assays and cell-based lipopolysaccharide (LPS)-induced nitric oxide (NO) inhibitory assays in RAW 264.7 murine macrophages. In addition, scavenging assays using 1,1-diphenyl-2-picrylhydrazyl (DPPH) radical, 2,2'-azino-bis(3-ethylbenzothiazoline-6-sulfonic acid) diammonium salt (ABTS) radical, and peroxynitrite ($ONOO^-$) were used to evaluate antioxidant potential and TPC was selected to assess phytochemical characteristics. Between the two kohlrabi cultivars, red kohlrabi (RK) had two times more TPC than green kohlrabi (GK) and showed significant antioxidant effects in DPPH, ABTS, and $ONOO^-$ scavenging assays. Likewise, methanol (MeOH) extracts of RK and GK inhibited LPS-induced NO production in a dose dependent manner that was further clarified by suppression of iNOS and COX-2 protein production. The MeOH extracts of RK and GK exhibited potent inhibitory activities against PTP1B with the corresponding $IC_{50}$ values of $207{\pm}3.48$ and $287{\pm}3.22{\mu}g/mL$, respectively. Interestingly, the RK MeOH extract exhibited significantly stronger anti-inflammatory, anti-diabetic, and antioxidant effects than that of GK MeOH extract. As a result, our study establishes that RK extract with a higher TPC might be useful as a potent anti-diabetic, antioxidant, and anti-inflammatory agent.

A useful material isolation from the Bombycis corpus (Beauveria bassiana) growing of the silkworm, Bombyx mori L. (백강잠(Bombysis corpus)으로부터 유용물질 분리)

  • 정이연;남성희;홍인표;유승헌;권학철;이강노
    • Journal of Sericultural and Entomological Science
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    • v.45 no.1
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    • pp.25-30
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    • 2003
  • This study was carried out to investigate active constituents of Bombysis corpus on the neurite outgrowth from PC 12 cells led to isolate three new and a known sphingolipids from the n-hexane soluble portion and five amines from the butanol soluble portion of its methanol extract. On the basis of spectroscopic data, their structures have been elucidated as (4E,6E,2S,3R)-2-N-eicosanoyl-4,6-tetradecasphingadienine, (4E,2S,3R)-2-N-eicosanoyl-4-tetradecasphingenine,(4E,6E,2S,3R)-2-N-docosanoyl-4,6-tetradecasphingadienine,(4E,6E,2S,3R)-2-N-docosanoyl-4,6-tetradecasphingadienine,(4E,2S,3R)-2-N-octadecanoyl-4-tetradecasphingenine, 1,7-dimethyl-xanthine, uracil, urea, betaine and tyrosine, respectively. The neurite outgrowth activities of these compounds were examined in PC12 cells by measuring the length of neurites. These compounds promoted neurite outgrowth in PC12 cells significantly.

Quality Characteristics of Dried Noodle Prepared with Doenjang Powder (된장분말을 첨가한 국수의 품질 특성)

  • Park, Bock-Hee;Koh, Kyeong-Mi;Cha, Min-hye;Kim, Sol;Jeon, Eun-Raye
    • Journal of the Korean Society of Food Culture
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    • v.31 no.6
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    • pp.616-623
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    • 2016
  • This study evaluated the quality characteristics of dried noodles prepared with doenjang powder (0, 3, 6, 9%) in order to determine the most preferred noodle recipe. The proximate composition of the composite doenjang powder-wheat flours was as follows: calories 365 kcal, carbohydrates 79 g, saccharides 3 g, protein 10 g, fat 0.8 g, and sodium 65 mg. As the level of doenjang powder increased, the content of total amino acids increased; aspartic acid, glutamic acid, tyrosine, and lysine contents increased, in particular. Water-binding capacity decreased as the level of doenjang powder increased. When viscosity of composite doenjang powder-wheat flours was measured by amylography, gelatinization point increased as the level of doenjang powder increased. As the level of doenjang powder increased, L value decreased, whereas a and b values increased. Weight, water absorption, and volume of cooked noodles decreased, whereas turbidity of soup increased. For textural properties, hardness decreased, whereas springiness increased as the level of doenjang powder increased. For overall preference according to the results of sensory evaluation, noodles added with 3% doenjang powder were the most preferred. According to the results, addition of doenjang powder positively affects the overall sensory evaluation of dried noodle, and 3% is the optimal level for addition.

Whitening improvement effect of Hermetia illucens larvae extracts (아메리카동애등에(Hermetia illucens) 유충 추출물의 미백개선 효과)

  • Park, Ji Yeong;Kim, Sun Young;Koo, Bonwoo;Kim, Eunsun;Kim, Yong-Soon;Park, Kwanho
    • Journal of Environmental Science International
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    • v.31 no.10
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    • pp.883-890
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    • 2022
  • The present study investigated the feasibility of using the ethanolic extract of Hermetia illucens larvae (HIE) as a whitening improvement material. In cell viability assays using B16F1 melanoma cells, no cytotoxicity was recorded up to 200 ㎍·mL-1 of HIE. Moreover, while tyrosinase inhibitory activity increased, melanin content decreased in a dose-dependent manner, indicating that HIE likely inhibited tyrosine-induced intracellular melanin biosynthesis in B16F1 melanoma cells. Therefore, HIE is expected to serve as a potent whitening improvement material.

Root Extract of Scutellaria Baicalensis Increases Gefitinib Sensitivity in H1975 Human Non-small Cell Lung Cancer Cells (H1975 세포에서 황금추출물에 의한 gefitinib 저항성 억제 효과)

  • Park, Shin-Hyung;Park, Hyun-Ji
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.35 no.4
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    • pp.117-123
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    • 2021
  • Gefitinib, a first generation epidermal growth factor receptor tyrosine kinase inhibitor (EGFR TKI), provides obvious clinical benefit in patients with EGFR-mutant non-small cell lung cancer (NSCLC). However, patients ultimately develop gefitinib resistance which mainly caused by EGFR T790M secondary mutation. In the current study, we investigated whether the root extract of Scutellaria baicalensis (SB) overcomes gefitinib resistance. Gefitinib-resistant H1975 human NSCLC cells (EGFR L858R/T790M double mutant) were treated with gefitinib and/or ethanol extract of SB (ESB) to evaluate the effect of ESB on the gefitinib sensitivity. The cell viability was measured by MTT assay and trypan blue exclusion assay. The colony-forming ability was evaluated by anchorage-dependent colony formation assay. Combined treatment with gefitinib and ESB markedly decreased the cell viability and colony formation than single treatment with gefitinib or ESB in H1975 cells. In addition, cells treated with both gefitinib and ESB exhibited a significant increase of sub-G1 DNA content which indicates apoptotic cells compared with those treated with gefitinib or ESB alone. As a molecular mechanism, combined treatment with gefitinib and ESB strongly downregulated the phosphorylation of ERK and JNK than single treatment with gefitinib or ESB. Taken together, our results demonstrate that ESB sensitizes H1975 cells to gefitinib treatment. We cautiously propose that ESB can be used in combination with gefitinib for the advanced NSCLC patients with acquired resistance to EGFR TKIs.