• 대한유전성대사질환학회지
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• 제14권1호
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• pp.42-47
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• 2014

Gaucher disease is a multisystemic disorder arising from a deficient activity of the lysosomal enzyme glucocerebrosidase, which leads to accumulation of glycosylceraide and other glycolipids in the regiculoendothelial system. The characteristics of Gaucher disease are anemia, thrombocytopenia, hepatosplenomegaly, and skeletal disease. Enzyme replacement therapy (ERT) has been proven to prevent progressive manifestations of Gaucher disease and effective in improving anemia, thrombocytopenia, bone markers and biomarkers. However, some patient needs still remain unmet because of the inaccessibility of certain sites including brain, bone and various organs. ERT could not Improve the irreversible lesion such as liver fibrosis, hepatopulmonary syndrome, and necrosis or infarction of bone and other organs. Adult patients with Gaucher disease should be screened for longterm complication such as bone disease, pulmonary hypertension, gallstone, and cancer, especially in patients with splenectomy. Parkinsonism and polyneuropathy was also reported among patients with type 1 Gaucher disease, but ERT does not improve neurological function. We need to review the benefits and unmet needs of ERT in Gaucher disease.

• 대한유전성대사질환학회지
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• 제14권1호
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• pp.37-41
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• 2014

Fabry disease is an X-linked disease caused by deficiency of the lysosomal enzyme alpha-galactosidase A. Affected males present anhydrosis, acroparesthesia and angiokeratoma, and subsequently cardiac, cerebral and renal complications are followed. Females and atypical variants show heterogeneous clinical symptoms. In 2001, two recombinant enzymes were approved for Fabry disease: agalsidase alpha and agalsidase beta. Since the introduction of enzyme replacement therapy (ERT), the number of long-term follow-up studies has been reported. Long-term ERT showed effectiveness on renal function in patients with chronic kidney disease, decrease or stabilization of left ventricular mass, and improvement of pain and quality of life. However, there were limited effects on cerebrovascular events and their mortality. Current literatures on the clinical effect of ERT have reported limited datain adult patients who have already advanced disease. Therefore, further study for pre-symptomatic patients and atypical variants is needed to verify the impact of ERT. This review summarized recent progresses in ERT and limitations of long-term effect of ERT in patients with Fabry disease.

• 한국철도학회:학술대회논문집
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• 한국철도학회 2005년도 춘계학술대회 논문집
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• pp.610-614
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• 2005

Rock mass classification by using electrical resistivity tomography(ERT) method is widely performed for the determination of rock support type in tunnel design. In the region of high electrical noise level, however, the result of the ERT will have many erroneous features. In this study, the back ground electrical noise had been measured to find out the reason why the results of ERT in this area did not agree to the expected geology confirmed by boreholes. In order to overcome this limitation of ERT, a hybrid surface-borehole array seismic refraction tomography had been followed. Using this technique, we could get P-wave velocity section including the depth level of tunnel. The comparison of the P-wave velocity and RMR shows fairly good statistical relationship to make it possible to set up the rock mass classification for the entire tunnel line.

• 한국환경복원기술학회지
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• 제18권1호
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• pp.153-163
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• 2015

The study was conducted to develop ecological restoration method of damaged area in DMZ vicinities. As the material for the ecological restoration, forest soil and trees waste have been used. Forest soils were collected for experiments in Yanggu, Gangwon Province. Effect of ecological restoration was analyzed through germination experiment. Germination experiment was performed using 12 kinds of woody and herbaceous seeds. Woody and herbaceous seed germination in test pot was relatively evenly. Mixed material consisting of forest soil and trees waste seemed a possibility as the material for the restoration. The effects on seed germination were higher in the case of mixing more than 70% by volume ratio of local resources. Total number germinated individuals were different depending on the mixing ratio of soil sampling depth. Individual plants showed different trends depending on the experimental combination. Results of the woody seed germination were affected only in the soil sampling depth. Seed germination of herbaceous received a combined effect on soil sampling depth and mixing ratio.

• 한국대기환경학회지
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• 제30권3호
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• pp.261-269
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• 2014

This study focused on the performance of the newly developed hybrid filter system to capture fine particulate matter and mercury compounds in a coal-fired power plant. The hybrid filter system combining bag-filter and electrostatic precipitator had been developed to remove fine particulate matter. However, it would have a good performance to control mercury compounds as well. In Hybrid filter capture system, the total removal efficiency of total mercury compounds consisting of particulate mercury ($Hg_p$), oxidized mercury ($Hg^{2+}$), and elemental mercury ($Hg^0$) was 66.2%. The speciation of mercury compounds at inlet and outlet of Hybrid filter capture system were 1.3% and 0% of $Hg_p$, 85.2% and 68.1% of $Hg^0$, and 13.5% and 31.9% of $Hg^{2+}$, respectively. In hybrid filter capture system injected with 100% of flue-gas, the removal efficiency of total mercury was calculated to increase to 93.5%.

• 한국콘텐츠학회논문지
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• 제22권1호
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• pp.431-443
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• 2022

본 연구는 코로나19 범유행 동안의 의료 면담 교육 지속을 위하여 화상 회의 방식의 긴급 비대면 수업(Emergency Remote Teaching:이하 ERT)을 도입한 일개 의과 대학의 사례를 보고하는 것이다. ERT 실습과 기존 면대면 실습과의 차이에 초점을 두고 표준화 환자 인터뷰를 통하여 수업 운영 경험을 기술하고 학생들의 학업성취도를 통계적으로 분석하여 교육 효과를 살펴보았다. 표준화 환자들은 면담 과정에서 원격 상대와 눈 맞춤을 하기 어려우며, 대화 시의 생동감과 재미가 다소 떨어지나, 대면 실습보다 대화 내용에 대한 집중도는 높은 느낌을 받았다는 의견을 내놓았다. 비대면 수업의 수강생들은 온라인으로 실시한 단원별 테스트에서 기존 면대면 수업 수강생과 비슷한 성적을 받았으나, 대면으로 치러진 기말고사에서는 유의하게 낮은 성적을 받았는데, 이러한 결과는 면대면 접촉과 비디오 매개 커뮤니케이션의 차이에서 기인한 것으로 해석된다. 본 연구의 사례가 비대면 의사-환자 커뮤니케이션의 중요성을 환기하고, 포스트 코로나 시대에 대비한 원격 의료 면담 훈련법 개발로 확장될 수 있기를 기대한다.

• Journal of Genetic Medicine
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• 제20권1호
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• pp.6-14
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• 2023

Fabry disease (FD), a rare X-linked lysosomal storage disorder, is caused by mutations in the α-galactosidase A gene gene encoding α-galactosidase A (α-Gal A). The functional deficiency of α-Gal A results in progressive accumulation of neutral glycosphingolipids, causing multi-organ damages including cardiac, renal, cerebrovascular systems. The current treatment is comprised of enzyme replacement therapy (ERT), oral pharmacological chaperone therapy and adjunctive supportive therapy. ERT has been introduced 20 years ago, changing the outcome of FD patients with proven effectiveness. However, FD patients have many unmet needs. ERT needs a life-long intravenous therapy, inefficient bio-distribution, and generation of anti-drug antibodies. Migalastat, a pharmacological chaperone, augmenting α-Gal A enzyme activity only in patients with mutations amenable to the therapy, is now available for clinical practice. Furthermore, these therapies should be initiated before the organ damage becomes irreversible. Development of novel drugs aim at improving the clinical effectiveness and convenience of therapy. Clinical trial of next generation ERT is underway. Polyethylene glycolylated enzyme has a longer half-life and potentially reduced antigenicity, compared with standard preparations with longer dosing interval. Moss-derived enzyme has a higher affinity for mannose receptors, and seems to have more efficient access to podocytes of kidney which is relatively resistant to reach by conventional ERT. Substrate reduction therapy is currently under clinical trial. Gene therapy has now been started in several clinical trials using in vivo and ex vivo technologies. Early results are emerging. Other strategic approaches at preclinical research level are stem cell-based therapy with genome editing and systemic mRNA therapy.

• 한국자원식물학회:학술대회논문집
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• 한국자원식물학회 2023년도 임시총회 및 춘계학술대회
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• pp.56-56
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• 2023

The cone of Pinus rigida × Pinus taeda (PRT), a plant in the Pinaceae family, has long been used in traditional medicine to treat hemostasis, bruises, and burns. Previous research has shown that regulating oxidation-reduction reactions in reactive oxygen species can help inhibit melanogenesis, the process of melanin synthesis, which is a common target for addressing hyperpigmentation. Inhibiting tyrosinase is also known to be effective in this regard. Based on these findings, we conducted an investigation into the inhibitory effect of the ethyl acetate fraction of PRT (ERT) on melanogenesis in B16 F10 cells. We know that the expression levels of melanin biosynthesis-related proteins, including tyrosinase, TRP-1, and TRP-2, are regulated by MITF (microphthalmia-associated transcription factor) and cAMP, with cAMP affecting the activity of protein kinase A (PKA). PKA can reduce melanogenesis, and CREB reduces the phosphorylation of melanin-producing enzymes. In addition, the MAPK signaling pathway, composed of ERK, JNK, p38, and other factors, is also known to play a role in the inhibition of melanogenesis in melanocytes. Our immunoblotting results showed that ERT inhibited the expression of melanin production-related proteins (tyrosinase, TRP-1, TRP-2, and MITF) that were significantly increased by a-MSH treatment to promote melanin production. Furthermore, the phosphorylation levels of factors related to cAMP/PKA/CREB and MAPK signaling pathways were significantly reduced without affecting the total form. In conclusion, we believe that treatment with ERT can inhibit melanin synthesis by modulating the phosphorylation of cAMP/PKA/CREB and MAPK signaling pathways at the cellular level. These findings suggest the potential of ERT as a raw material for functional cosmetics and pharmaceuticals, thanks to its antioxidant activity and ability to inhibit melanogenesis. We thought that these findings of ERT as a natural plant resource will inspire further research and development in this area.

• Journal of mucopolysaccharidosis and rare diseases
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• 제4권1호
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• pp.11-13
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• 2018

In Malaysia, diagnosis and treatment of patients with mucopolysaccharidoses (MPS) is mainly localized at Hospital Kuala Lumpur, which is the national referral center for rare diseases. To date there are 83 patients diagnosed with MPS in our center, with MPS II being the commonest. The Malaysian National Medicines Policy second edition has a specific section on the orphan drugs which includes recombinant human enzyme for enzyme replacement therapy (ERT) in MPS. So far, National Pharmaceutical Regulatory Agency Malaysia has approved recombinant human enzyme for MPS types I (Loranidase), II (idursulfase), IVA (elosulfase alfa), and VI (Galsufase). Access to Idursulfase beta (another recombinant human enzyme for MPS II) and vestronidase alfa-vjbk (MPS VII) required special authorization on named patient basic. Currently there are 25 patients receiving ERT, 70% of the funding are from Ministry of Health (MOH), the remaining 30% are from various charitable funds and humanitarian programs. Thirteen newly diagnosed patients have to queue for an additional fund. Four patients have been treated with Hematopoietic stem cell transplant. MOH has also published guidelines regarding the patient selection criteria for ERT and treatment monitoring schedule.

• International Journal of Fuzzy Logic and Intelligent Systems
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• 제6권1호
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• pp.47-51
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• 2006

Electrical resistance tomograpy (ERT) maps resistivity values of the soil subsurface and characterizes buried objects. The characterization includes location, size, and resistivity of buried objects. In this paper, truncated least squares (TLS) is presented for the solution of the ERT image reconstruction. Results of numerical experiments in ERT solved by the TLS approach is presented and compared to that obtained by the Gauss-Newton method.