• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권5호
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• pp.479-486
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• 2019

Progressive familial intrahepatic cholestasis (PFIC) is a group of severe genetic disorders, inherited in an autosomal recessive manner, causing cholestasis of hepatocellular origin, later progressing to biliary cirrhosis and liver failure. This is the first report of PFIC type 1 with novel compound heterozygous mutations in Korea. The patient was presented with intrahepatic cholestasis, a normal level of serum ${\gamma}-glutamyl$ transferase, steatorrhea, and growth failure. Genetic testing of this patient revealed novel compound heterozygous mutations (p.Glu585Ter and p.Leu749Pro) in the ATP8B1 gene. After a liver transplantation at age 19 months, the patient developed severe post-transplant steatohepatitis.

• 한국응용약물학회:학술대회논문집
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• 한국응용약물학회 1996년도 춘계학술대회
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• pp.219-219
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• 1996

Mucin release from hamster tracheal surface epithelial(HTSE) cells can be stimulated by extracellular ATP via activation of P$_2$ purinoceptors located on the cell surface which appears to be coupled to phospholipase C via G proteins. However, our preliminary data indicate that the ATP-induced mucin release involves, in part, activation of PKC, but not an increase in the intracellular Ca++ level, suggesting the presence of another pathway which is separate from the PLC-PKC pathway, In this study, we intended to confirm the previous observation and subsequently identify an additional mechanism. Confluent HTSE cells were metabolically labeled with either $^3$H-glucosamine or $^3$H-arachidonic acid(AA), and release of either $^3$H-mucin or $^3$H-AA was quantified following various treatments. $^3$H-mucin was assayed using the sepharose CL-4B gel-filtration method, whereas $^3$H-AA liberation was measured by counting $^3$H-radioactivity in the chase medium. We found that: (1)Desensitization of PKC by pretreatment with PMA completely abolished the mucin releasing effect of PMA but partially inhibited the ATP-induced mucin release; (2) ATP increases release of $^3$H-AA in a dose-dependent fashion; (3) mepacrine, an inhibitor of PLA$_2$, attenuates ATP-induced mucin release in a dose-dependent fashion. These results confirm our previous notion that the PLC-PKC pathway is responsible, in part, for ATP-induced mucin release. Furthermore, activation of PLA$_2$ appears to be an additional pathway which is involved in ATP-induced mucin release.

• International Journal of Industrial Entomology and Biomaterials
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• 제15권2호
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• pp.115-122
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• 2007

As the genome of B.mori is available in GenBank and the EST database of B.mori is expanding, identification of novel genes of B.mori is conceivable by data-mining techniques. We used the in silico cloning method to get the vacuolar-type $H^+-ATP$ synthetase (V-ATPase) c subunit (16 kDa proteolipid subunit) gene of B.mori and analysed with bioinformatics tools. The result was confirmed by RT-PCR and sequencing. The V-ATPase c subunit cDNA contains a 468 bp ORF. The ORF encoded a 155-residue protein that showed extensive homology with V-ATPase c subunits from other 15 species and contained four membrane-spanning helices. Tissue expression pattern analysis revealed that V-ATPase c expressed strongly in Malpighian tubules, not in fat body. This gene has been registered in GenBank under the accession number EU082222.

• Journal of Chest Surgery
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• 제13권1호
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• pp.21-25
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• 1980

This study was attempted to evaluate the effectiveness of cold cardioplegic solution [group B] compared with that of intermittent aortic cross clamping [group A]. 1. In group A, two dogs recovered spontaneous heart beat without using defibrillator and one case was used defibrillator once. In group B, four dogs recovered the spontaneous heart beat even without defibrillator and in one animal the defibrillator was used three times. 2. There was no significant difference in serum electrolyte [Na+, Ca2+, Cl- ] between group, but K+ level was decreased in both group. 3. The group A showed more increasing in serum LDH activity than group B. 4. The postoperative myocardial ATP level was $2.35{\pm}0.10$ ${\mu}$M/Gm. of wet weight in control group, $1.46{\pm}0.16$${\mu}$M/Gm. of wet weight in group A [60.2% of control group], and $1.80{\pm}0.14$${\mu}$M/Gm. of wet weight in group B [76.8% of control group].

• Journal of Plant Biology
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• 제23권1호
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• pp.17-25
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• 1980

Kidneybean(Phaseolus vlgaris L.) and buckwheat(Fagopyrum esculentum M$\"{O}$nch) seedlings cultured with Hoagland solution to the height of 7 to 10cm in the earthern pot containing sand were used for experimental plants. One group of the plants was irrigated with Hoagland solution composed of various Al concentrations of 0 to 1,000 ppm containing 50ppm Ca, and the other group was irrigated with Hoagland solution composed of various Ca concentrations of 0 to 1,000ppm containing 100ppm Al for a month during June and July. By Al-surplus over 100ppm, the margins of backwheat leaves were curied down and turned into yellow-brown necrosis simialr to Ca-deficiency, and the old kidney bean leaves showed marginal and veinal chlorosis in addition to the early shedding of leaves. With Al-surplus of 100ppm the content of chlorophyll a and b in both plants was decreased by 30~40%. The ratio 3/1 of chlorophyll a/b was not altered by Al-surplus and Ca-deficienty at an early stage, but it was affected markedly at a final stage of growth. Leaf respiration with 100ppm Al containing 50pm Ca was slightly affected in buckwheat, but decreased by 20% in kidneybean. With Al-surplus of 100ppm the ATP content was decreased by 32% in the kidneybean leaves and by 80% in buckwheat leaves, whereas with Ca-deficiency it was decreased by 72% in kidneybean and by 90% in buckwheat. The rhythm of ATP level showed self-relience without being affected by light or temperature under the green house condition although by Al-s rplus and Ca-deficiency the ATP level of the rhythm was lower than that of control.trol.

• Journal of Yeungnam Medical Science
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• 제12권2호
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• pp.246-259
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• 1995

배뇨근의 운동성에 대한 baclofen의 억제작용의 기전을 탐구하기 위하여 다음과 같은 실험을 하였다. 흰쥐 (Sprague-Dawley)의 방광에서 적출한 배뇨근절편을 적출근편실험조에 현수하고, 등척성 장력측정기를 사용하여 그 수축력을 묘기하였다. 실험조내의 영양액의 온도는 $37^{\circ}C$로 유지시키고, 95% 산소와 5% 이산화탄소의 혼합기체를 공급하여 pH를 7.4로 유지하였다. 배뇨근절편은 전기장자극에 의해 수축하였는데, 8분간의 전기장자극 유발수축 중 최초의 급격한 수축반응은 mATP와 baclofen에 의해 약간 억제되는 경향을 보였으며, 후기 4분간의 수축반응은 콜린성 무스카린성 수용체 봉쇄제인 atropine과 $GABA_B$ 수용체 효현제인 baclofen에 의해 유의하게 억제되었다. Atropine은 배뇨근 절편의 acetylcholine 유발 수축을 길항하였고, mATP는 ATP 유발 수축을 완전히 봉쇄하였으나, baclofen 존재하에서는 acetylcholine이나 ATP 첨가에 의한 배뇨근의 수축이 영향을 받지 않았다. 이상의 결과를 종합하면, 흰쥐 방광에는 $GABA_B$ 수용체가 존재하며 baclofen은 이 수용체를 통하여 콜린성신경 말단에서의 신경전달체의 유리를 억제하여 배뇨근의 수축성을 감소시킨다고 사료된다.

• Journal of Microbiology and Biotechnology
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• 제23권6호
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• pp.818-825
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• 2013

We isolated and functionally characterized the ${\alpha}$- and ${\beta}$-subunits (ApCpnA and ApCpnB) of a chaperonin from Aeropyrum pernix K1. The constructed vectors pET3d-ApCpnA and pET21a-ApCpnB were transformed into E. coli Rosetta (DE3), BL21 (DE3), or CodonPlus (DE3) cells. The expression of ApCpnA (60.7 kDa) and ApCpnB (61.2 kDa) was confirmed by SDS-PAGE analysis. Recombinant ApCpnA and ApCpnB were purified by heat-shock treatment and anion-exchange chromatography. ApCpnA and ApCpnB were able to hydrolyze not only ATP, but also CTP, GTP, and UTP, albeit with different efficacies. Purified ApCpnA and ApCpnB showed the highest ATPase, CTPase, UTPase, and GTPase activities at $80^{\circ}C$. Furthermore, the addition of ApCpnA and ApCpnB effectively protected citrate synthase (CS) and alcohol dehydrogenase (ADH) from thermal aggregation and inactivation at $43^{\circ}C$ and $50^{\circ}C$, respectively. In particular, the addition of ATP or CTP to ApCpnA and ApCpnB resulted in the most effective prevention of thermal aggregation and inactivation of CS and ADH. The ATPase activity of the two chaperonin subunits was dependent on the salt concentration. Among the ions we examined, potassium ions were the most effective at enhancing the ATP hydrolysis activity of ApCpnA and ApCpnB.

• Ocean and Polar Research
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• 제35권1호
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• pp.39-50
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• 2013

Biomass and community composition of microphytobentos in tidal flats were studied by HPLC analysis and also investigated to examine the relationship between microphytobenthic pigments and Adenosine-5' triphosphate (ATP) as an index of total microbial biomass in intertidal environments (muddy and sandy sediment) of Gyeonggi Bay, west coast of Korea. Microphytobenthic pigments and ATP concentration in muddy sediment were the highest at the surface while the biomass of microphytobenthos in sandy sediment was the highest at the sub-surface (0.75 cm sediment depth). The detected pigments of microphytobenthos were chlorophyll a, b (euglenophytes), $c_3$, peridinin (dinoflagellates), fucoxanthin (diatom or chrysophytes), diadinoxanthin, alloxanthin (cryptophytes), diatoxanthin, zeaxanthin (cyanobacteria), ${\beta}$-carotein, and pheophytin a (the degraded product of chlorophyll a). Among the pigments which were detected, the concentration of fucoxanthin was the highest, indicating that diatoms dominated in the microphytobenthic community of the tidal flats. There was little significant correlation between OC (Organic Carbon) and ATP in both sediments. However, a positive correlation between chlorophyll a concentration and ATP concentration was found in sandy sediment, suggesting that microbial biomass could be affected by labile OC derived from microphytobenthos. These results provide information that may help us understand the relationship between microphytobenthos and microbial biomass in different intertidal sediment environments.

• 토지주택연구
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• 제3권3호
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• pp.299-305
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• 2012

음식물쓰레기 발효 소멸용 목질바이오칩의 종류별로 세공구조에 따른 음식물쓰레기 무게 감량율 및 미생물 활동성을 비교분석 하였다. 목질바이오칩을 이용한 음식물쓰레기 발효 소멸실험을 온도 $30{\sim}50^{\circ}C$, 습도 30~70% 조건의 발효 소멸 반응조에 15일간 매일 700~1,500g의 음식물쓰레기를 투입하며 실시하였다. 이 때 1,500g의 목질바이오칩을 발효 소멸 반응조에 초기에 투입하였다. 실험에 사용한 목질바이오칩의 세공구조는 미생물 혼합형(A 바이오칩), $2{\mu}m$ 마크로 세공형(B biochip), $0.1{\mu}m$ 미세공형(C 바이오칩), 점성구조형(D 바이오칩)으로 4가지 유형이었다. 실험결과, A, B, C, D 바이오칩별 발효 소멸에 의한 음식물쓰레기 무게감량율은 각각 85%, 63%, 92%, 73%이었고, C 바이오칩의 경우가 음식물쓰레기 감량율 92%로 최고값을 나타내었다. 또한, C 바이오칩은 ATP/COD $3.00{\times}10^{-10}$, ATP/TN $2.31{\times}10^{-11}$로 상대적으로 타 종류의 바이오칩보다 높은 결과를 나타내었다. 이는 발효 소멸반응에서 발생되는 미생물의 서식지를 충분히 제공하여 ATP/COD 및 ATP/TN이 높아졌고 미생물의 활동성이 강화되어 발효 소멸반응이 원활하게 진행된 결과에 기인하는 것으로 분석되었다.

• Asian-Australasian Journal of Animal Sciences
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• 제25권2호
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• pp.194-199
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• 2012

Liver and pulmonary artery tissue from 5 Angus cross bred steers, 6 goats, and 6 pigs were collected at a commercial abattoir to examine the relationship of pulmonary artery copper (Cu) concentrations and genes involved in copper homeostasis. Liver and pulmonary artery samples were collected at the time of harvest and snap frozen. Liver and pulmonary artery Cu concentrations were determined via flame atomic absorption spectrophotometry and gene expression was determined via real time PCR. Liver Cu concentrations (mg Cu/kg DM${\pm}$SE) were higher (p<0.01) in cows ($396.4{\pm}109.1$) and goats ($181.4{\pm}37.0$) than in pigs ($19.2{\pm}3.5$). All liver Cu concentrations were within normal ranges and considered adequate for each species. Liver Cu concentration was more variable in cows and goats compared to pig liver Cu concentrations. Pulmonary artery ${\beta}$-hydroxylproline was higher (p<0.01) in cow and pig than goat. Real Time PCR revealed that goat liver atp7a was positively correlated ($r^2$ = 0.92; p<0.01) to liver Cu concentrations while cow and pig atp7a was not correlated to liver Cu concentration. In the pig, liver atp7a concentration was positively correlated to atp7b ($r^2$ = 0.66; p<0.05). Pulmonary artery Cu concentration was highest in cows ($14.9{\pm}4.7$), intermediate in pigs ($8.9{\pm}3.3$), and lowest in goats ($3.9{\pm}1.1$). Goat pulmonary artery Cu concentration was not correlated to ctr1 concentration, however, atp7a concentration was positively correlated with ctr1 ($r^2$ = 0.90; p<0.01). In cow pulmonary artery, loxl1 concentration was positively correlated to eln mRNA concentration ($r^2$ = 0.91; p<0.02). Pulmonary artery CTR1 protein concentration was positively correlated to pulmonary artery Cu ($r^2$ = 0.85; p = 0.03) concentration while negatively correlated to liver Cu ($r^2$ = -0.79; p<0.04). Pulmonary artery Cu concentration was not correlated to concentration of Cu homeostatic genes in the pig. These data indicate that genes involved in Cu homeostasis (ctr1, atp7A, atp7B, loxl1 and eln) are differently regulated in different species.