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      • KCI등재

        Long-Term Efficacy and Safety of Eculizumab in Patients With Paroxysmal Nocturnal Hemoglobinuria and High Disease Burden: Real-World Data From Korea

        Kim Jin Seok,Jang Jun Ho,Jo Deog-Yeon,Ahn Seo-Yeon,Yoon Sung-Soo,Lee Je-Hwan,Kim Sung-Hyun,Choi Chul Won,Shin Ho-Jin,Kim Min-Kyoung,Lee Jae Hoon,Mun Yeung-Chul,Kong Jee Hyun,Hyun BokJin,Nam HyunSun,Ki 대한의학회 2023 Journal of Korean medical science Vol.38 No.41

        Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder characterized by uncontrolled terminal complement activation. Eculizumab, a monoclonal antibody C5 inhibitor was introduced in Korea in 2009 and has been the standard treatment option for PNH. Methods: This study assessed the long-term efficacy/safety of eculizumab in PNH using real-world data from the Korean Health Insurance Review and Assessment Service. Eighty patients who initiated eculizumab from 2009–2020 were enrolled. Results: At eculizumab initiation, the median age was 51.5 years, lactate dehydrogenase (LDH) 6.8 × upper limit of normal, and granulocyte clone size 93.0%. All patients had at least one PNH-related complication before eculizumab initiation, including renal failure (n = 36), smooth muscle spasm (n = 24), thromboembolism (n = 20), and pulmonary hypertension (n = 15). The median (range) duration of eculizumab treatment was 52.7 (1.0, 127.3) months (338.6 total treated patient-years). Despite high disease activity in the study population before treatment initiation, overall survival was 96.2% and LDH levels were stabilized in most patients during treatment. PNH-related complications at treatment initiation were resolved in 44.4% of patients with renal failure, 95.8% with smooth muscle spasm, 70.0% with thromboembolism, and 26.7% with pulmonary hypertension. Extravascular hemolysis occurred in 28.8% of patients (n = 23; 0.09 per patient-year) and breakthrough hemolysis in 18.8% (n = 15; 0.06 per patient-year). No treatment discontinuation cases related to eculizumab were observed. Conclusion: These data provided evidence for the long-term efficacy and safety of eculizumab in Korean PNH patients with high disease burdens.

      • KCI등재

        Gain of a New Exon by a Lineage-Specific Alu Element-Integration Event in the BCS1L Gene during Primate Evolution

        Park, Sang-Je,Kim, Young-Hyun,Lee, Sang-Rae,Choe, Se-Hee,Kim, Myung-Jin,Kim, Sun-Uk,Kim, Ji-Su,Sim, Bo-Woong,Song, Bong-Seok,Jeong, Kang-Jin,Jin, Yeung-Bae,Lee, Youngjeon,Park, Young-Ho,Park, Young Il Korean Society for Molecular and Cellular Biology 2015 Molecules and cells Vol.38 No.11

        BCS1L gene encodes mitochondrial protein and is a member of conserved AAA protein family. This gene is involved in the incorporation of Rieske FeS and Qcr10p into complex III of respiratory chain. In our previous study, AluYRa2-derived alternative transcript in rhesus monkey genome was identified. However, this transcript has not been reported in human genome. In present study, we conducted evolutionary analysis of AluYRa2-exonized transcript with various primate genomic DNAs and cDNAs from humans, rhesus monkeys, and crabeating monkeys. Remarkably, our results show that AluYRa2 element has only been integrated into genomes of Macaca species. This Macaca lineage-specific integration of AluYRa2 element led to exonization event in the first intron region of BCS1L gene by producing a conserved 3' splice site. Intriguingly, in rhesus and crabeating monkeys, more diverse transcript variants by alternative splicing (AS) events, including exon skipping and different 5' splice sites from humans, were identified. Alignment of amino acid sequences revealed that AluYRa2-exonized transcript has short N-terminal peptides. Therefore, AS events play a major role in the generation of various transcripts and proteins during primate evolution. In particular, lineage-specific integration of Alu elements and species-specific Alu-derived exonization events could be important sources of gene diversification in primates.

      • KCI등재

        넙치 근육 및 근소포체 미세구조의 변화에 미치는 전기자극의 영향

        김태진(Tae Jin Kim),김영진(Young Jin Kim),윤호동(Ho Dong Yoon),조영제(Toung Je Cho),최영준(Yeung Joon Choi),이근우(Keun Woo Lee),김건배(Geon Bae Kim),김동수(Dong Su Kim) 한국수산과학회 1998 한국수산과학회지 Vol.31 No.4

        Electron micrographs of muscle of plaice killed instantly by spiking at the head clearly showed the A-band, I-band, Z-line and M-line in muscle strips, whereas these bands could not be distinguished from each other in electrically stimulated plaice muscle stripes. As the electrical stimulation 7me increased, the continuity of Z-line disrupted rapidly. Electron microscopic observation skewed that sarcoplasmic reticulum (SR) in unstimulated plaice had natural triad structures between Z-line, However these structures were disrupted in the electrically stimulated sample. These structures were clearly observed after storage far 10 hrs at 5℃ from all the samples. In association with the Ca^(2+) translocation, Ca^(2+) -pyroantimonate deposits were observed at the inner part of SR immediately after spiked the plaice, However, Ca^(2+) -pyroantimonate deposits in electrically stimulated plaices were observed in the muscle strips and this phenomenon was clearly observed when electrical stimulation was prolonged.

      • SCISCIESCOPUS

        The Derived Neutrophil-to-Lymphocyte Ratio Is an Independent Prognostic Factor in Transplantation Ineligible Patients with Multiple Myeloma

        Lee, Gyeong-Won,Park, Sung Woo,Go, Se-Il,Kim, Hoon-Gu,Kim, Min Kyoung,Min, Chang-Ki,Kwak, Jae-Yong,Bae, Sang-Byung,Yoon, Sung-Soo,Lee, Je-Jung,Kim, Ki Hwan,Nam, Seung-Hyun,Mun, Yeung-Chul,Kim, Hyo Jun S.Karger 2018 Acta haematologica Vol.140 No.3

        <P><B><I>Background:</I></B> The neutrophil-to-lymphocyte ratio (NLR) is an independent prognostic marker in solid and hematological cancers. While the derived NLR (dNLR) was shown to be non-inferior to the NLR in large cohorts of patients with different cancer types, it has not been validated as a prognostic marker for multiple myeloma (MM) to date. <B><I>Methods:</I></B> Between May 22, 2011 and May 29, 2014, 176 patients with MM from 38 centers who were ineligible for autologous stem cell transplantation were analyzed. The dNLR was calculated using complete blood count differential data. The optimal dNLR cut-off value according to receiver operating characteristic analysis of overall survival (OS) was 1.51. All patients were treated with melphalan and prednisone combined with bortezomib. <B><I>Results:</I></B> The complete response rate was lower in the high dNLR group compared to the low dNLR group (7 vs. 26.1%, respectively; <I>p</I> = 0.0148); the corresponding 2-year OS rates were 72.2 and 84.7%, respectively (<I>p</I> = 0.0354). A high dNLR was an independent poor prognostic factor for OS (hazard ratio 2.217, 95% CI 1.015–4.842; <I>p</I> = 0.0458). <B><I>Conclusion:</I></B> The dNLR is a readily available and cheaply obtained parameter in clinical studies, and shows considerable potential as a new prognostic marker for transplantation-ineligible patients with MM.</P>

      • KCI등재

        넙치(Paralichthys olivaceus) 육의 사후 조기 변화 : 4 . 치사 방법이 육의 사후 경직도와 파괴 강도에 미치는 영향 4 . Effect of Killing Methods on Rigor Index and Breaking Strength of Muscle

        김재현,조영제,최영준,이근우,김육용,이남걸,김건배 한국수산학회 1994 한국수산과학회지 Vol.27 No.1

        넙치를 시료로 치사 방법(즉살, 고민사, 마취사 및 전기 자극사)을 달리하여 치사시킨 후에 저장하면서 치사 조건이 사후 조기의 어육의 사후 경직도와 파괴 강도의 변화에 미치는 영향을 검토한 결과를 요약하면 다음과 같다. 1. 사후 경직의 개시 시간은 전기 자극사와 고민사에서 현저히 단축되었고, 경직 개시 시간과 완전 경직 도달 시간은, 각각 전기 자극사일때 0시간과 9시간, 고민사일때 0시간과 13시간, 즉살일때 10시간과 36시간, 그리고 마취사일때 20시간과 56시간이었다. 2. 파괴 강도가 최대값에 도달하는 시간과 그때의 값은 각각 전기 자극사일때 치사 직후에 1,230.60±30.32g, 고민사일때 2.5시간과 1,235.83±35.37g 즉살일때 10시간과 1,186.29±55.90g, 그리고 마취사일때 15시간과 1,189.67±50.32g으로, 전기자극사와 고민사일때가 최대값이 높았고 그 저하 속도도 빨랐으며, 마취사일때는 파괴 강도가 최대로 되는 시간이 연장되었다. 3. 어체의 사후 경직도와 어육의 파괴 강도 사이에는 상관 관계가 없었으며, 사후 경직 개시기를 전후하여 파괴 강도가 가장 높았다. 이상의 결과로부터, 활어를 전기 자극사시킴으로서 치사 직후에 육질을 단단하게 만들 수 있을 뿐만 아니라, 또 마취사시키므로서 육질이 단단해지는 현상의 발생을 지연시킬 수 있으므로, 이런 방법들은 회맛의 향상 및 즉살 활어의 수송에 응용 가능할 것으로 사료된다. This study was undertaken to clarify the effect of killing methods on physical and rheological changes of plaice, Payalichthys olivaceus muscle at early period after death. Plaices killed by the four different methods(1. spiking at the brain instantly. 2.drowning in air. 3. dipping in 1,000ppm ethylaminobenzoate dissolved sea water as an anesthetic. 4. electrifying in sea water.) were stored at 5℃, and the rigor-index and breaking strength through storage were monitored. The longest onset time of rigor-mortis and full rigor was in the samples killed by dipping in sea water with dissolved anesthetic among all samples, where rigor-mortis began at 20hrs after killing and maximum tension was attained after 56hrs. However, in the cases of plaice electrified in sea water or drowned in air, the onset of rigor-mortis began just after killing and maximum tensions were attained after 9hrs and 13hrs, respectively. The level of breaking strength in the muscle of fish killed by spiking the brain instantly was 950.30 ±50.23g, immediately after killing. The value and time reached around the maximum breaking strength for each of the samples were 1,230.60±30.32g and Ohr(immediately after killing) for samples killed by electrifying in sea water, 1,235.83±35.37g and 2.5hrs for drowning samples, 1,186.29±55.90g and 10hrs for spiking samples, and 1,189.67±50.32g and 15hrs for samples dipped in anesthetic, respectively. From the results above, it could be concluded that electrification in sea water is the most effective method in accelerating rigor-mortis and shortening times of reaching the maximum breaking strength of fresh plaice flesh of all the killing methods at early periods after death, whereas dipping in sea water treated with anesthetic was the most effective way in delaying those changes.

      • KCI등재

        넙치(Paralichthys olivaceus) 육의 사후조기변화 : 6 . 치사 방법이 근원섬유의 형태학적 및 육의 조직학적인 변화에 미치는 영향 6 . Effect of Killing Methods on Morphological Changes of Myofibrills and Histological Changes of Muscle

        김재현,조영제,최영준,이근우,김육용,이남걸,김근배 한국수산학회 1994 한국수산과학회지 Vol.27 No.4

        치사 방법을 달리하여 치사한 넙치육을 5℃에서 저장하면서 치사 방법이 육의 파괴강도의 변화에 미치는 원인을 규명하기 위하여, 근원섬유의 형태학적인 변화 그리고 근육의 조직학적인 변화에 대하여 검토한 결과를 요약하면 다음과 같다. 1. 육의 파괴강도의 상승은 전기 자극사에서 가장 빠르고 최대값도 높았으며, 마취사에서 파괴강도가 최대로 되는 시간이 가장 연장되었다. 2. 근원섬유의 형태학적인 변화는 즉살 직후에는 근절내의 A대, H대, I대 그리고 Z선이 확실히 구별되었으나, 즉살한 시료는 저장 10시간후에 A대 중앙부의 H대 구별이 확실하지 알았으며, I대 간격이 좁아졌음이 관찰되었다. 마취사시킨 시료는 저장 15시간후에도 A대 중앙부의 H대가 희미하게 관찰되었다 한편, 전기 자극사시킨 시료는 치사 직후에 A대 중앙부의 H대의 구별이 불가능하였으며 I대의 간격이 좁아져 있었다. 3. 근육의 조직학적인 변화는, 즉살 직후에는 세포와 세포의 간격이 전혀 관찰되지 않았으나, 즉살 및 마취사에서는 각각 저장 15시간 및 24시간 후에 전체 세포 사이의 간격이 관찰되었다. 한편, 전기 자극사에서는 치사 직후에 세포 사이에 약간의 간격이 관찰되었을 뿐만 아니라. 저장 2.5시간후에 전체의 세포 사이에 간격이 넓어졌다. 이상의 결과로부터, 사후 조기에 육의 파괴강도의 변화는 myosin과 actin의 결합에 의한 장력의 발생 및 세포의 matrix구조의 파괴와 깊은 관계가 있음을 알 수 있다. This study was undertaken to clarify the effect of killing methods on the morphological and histological changes of plaice, Paralichthys olivaceus muscle at early stage after killing. Killed samples by the three different methods were stored at 5℃, and the changes in breaking strength of muscle, morphological observation of myofibrils and histological observation of extracellular spaces through storage were monitored. Samples killed by electrifying in sea water showed the maximum value of breaking strength immediately after killing and then it dropped significantly(p$lt;0.05) until 2.5hrs passed. Breaking strength of samples killed by spiking at the head instantly and dipping in sea water including anesthetic rose steadily over 10hrs and 15hrs after killing, respectively. In myofibrills prepared from dorsal muscles immediately after spiking at the head instantly, A-band, H-band, I-band, and Z-line in sarcomere were clearly distinguishable each other. Due to muscle contraction by electrical stimulation, it was impossible to distinguish H-band from I-band observed in sarcomere immediately after killing for samples killed by electrifying. But, in the cases of samples killed by spiking and dipping, H-band could be observed dimly until 10hrs and 15hrs storage. No extracellular space was observed among muscle cells immediately after spiking at the head instantly. Samples killed by spiking at the head instantly and dipping in sea water including anesthetic showed extracellular spaces among all muscle cells after 15hrs and 25hrs storage, respectively. The other hand, samples killed by electrifying in sea water(110V, 30sec.) showed a few extracellular spaces immediately after killing and then it showed extracellular spaces among all muscle cells after 2.5hrs storage.

      • KCI등재

        산 및 알칼리 공정으로 조제한 수리미 가열 겔의 물성 특성

        최영준(Yeung Joon Choi),박주동(Joo Dong Park),조영제(Young Je Cho),(Jae W. Park),김진수(Jin Soo Kim) 한국수산과학회 2002 한국수산과학회지 Vol.35 No.4

        알칼리 pH에서 어육 단백질을 녹인 후 등전점 처리를 통하여 단백질을 회수하고, 중성으로 pH를 조절하여 조제한 알칼리 수리미 가열 겔의 파괴강도와 변형값은 수세 과정을 통해 조제한 기존의 수리미 가열 겔에 비하여 손색이 없는 것으로 나타났으나, 백색도 값은 수리미에 남아있는 혈색소와 육 색소로 인하여 낮게 나타났다. 산처리 수리미의 저장 modulus 값은 수분 함량이 감소함에 따라 감소하였으나, 알칼리 수리미의 경우는 증가하였다. 수리미의 최종 pH는 저장 modulus 값에 영향을 미치고 형태는 산처리와 알칼리 처리 수리미 간에 다소 차이를 보였다. Potassium bromide는 S-S 결합의 형성을 통해 가열 겔의 저장 modulus를 증가시키는 것으로 나타났다. 알칼리 처리 수리미는 파괴강도, 변형값 및 백색도에 미루어 어묵 grade의 연제품 제조를 위한 원료로는 손색이 없는 것으로 판단하였다. Rheological properties of surimi gel from white fishes by acid (add surimi) and alkali (alkali surimi) process and effect of chemicals on gelation were investigated by punch and dynamic tests. The breaking force and deformation values of heat-induced gel of acid surimi were less than their values of alkali and conventional surimi gel, and whiteness was greatly decreased. Gel point of acid surimi was decreased but it of alkali surimi was increased with increasing moisture content in the range of 80 to 85%. Storage modulus of acid surimi was the highest vaule in pH 6.8, but that of alkali surimi showed high vaule at neutral and slightly alkali pH. Propylene glycol increased storage modulus in 20∼50℃, but urea and 2-mercaptoethanol suppressed it. Potassium bromide improved storage modulus in 20∼80℃. The results suggest that alkai process is used for making surimi instead of conventional method.

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