• Journal of mucopolysaccharidosis and rare diseases
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• 제3권1호
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• pp.1-8
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• 2017

Mucopolysaccharidosis (MPS) type III, or Sanfilippo syndrome is a rare autosomal recessive lysosomal storage disorder. It is caused by a deficiency of one of four enzymes involved in the degradation of the glycosaminoglycan (GAG) heparan sulfate. The resultant cellular accumulation of heparan sulfate causes various clinical manifestations. MPS III is divided into four subtypes depending on the deficient enzyme: MPS IIIA, MPS IIIB, MPS IIIC and MPS IIID. All the subtypes show similar clinical features and are characterized by progressive degeneration of the central nervous system (CNS). Main purpose of the treatment for MPS III is to prevent neurologic deterioration. However, conventional enzyme replacement therapy has a limitation due to inability to cross the blood-brain barrier. Several experimental treatment options for MPS III are being developed.

• 대한전자공학회논문지SD
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• 제40권6호
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• pp.393-399
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• 2003

일반적인 MPS 정류기의 순방향 전압강하 및 전력손실을 향상시키기 위해 몰리브덴을 장벽금속으로 사용하여 새로운 Mo-MPS 정류기를 제조하였다. 제조 된 Mo-MPS 정류기의 전기적 특성을 Al-MPS 및 Pt-MPS 정류기와 비교함으로써 특성을 평가하였다. 실험 결과 동일한 0.1A의 전류에서 Mo-MPs 정류기의 순방향 전압강하가 Al-MPS 및 Pt-MPS 정류기와 비교하여 각각 0.11V, 0.24V 낮게 나타났다. 따라서 순방향 전류밀도와 순방향 전압강하에 지배적으로 의존하는 전력손실에 있어서도 일반적인 MPS 정류기와 비교하여 향상되었다. 68% 쇼트키 접합 면적비를 가지는 Mo-MPS 정류기의 역방향 항복전압은 대략 304V로 나타났다. 이는 Al-MPS 및 Pt-MPS 보다 낮은 순방향 전압강하를 가지면서도 높은 역방향 항복전압 특성을 보여준다.

• Journal of mucopolysaccharidosis and rare diseases
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• 제1권1호
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• pp.2-4
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• 2015

Though the rate of incidence of each rare disease, including mucopolysaccharidosis (MPS), is low, this is not the case if they are taken as a whole. Rare diseases often have genetic causes and vary in type. However, the signs and symptoms vary greatly by disease, making it difficult to make accurate diagnoses and conduct necessary research, which is why we believe it is a field that deserves more attention and research. It is important to establish an infrastructure of experts in each country and promote cooperation within the Asia-Pacific region in order to improve specialist training and communication. Given the need for a system of cooperation, the Asia Pacific MPS Network (APMN) was established by several MPS experts in South Korea, Japan, and Taiwan in January 2013. Thereafter, the Asia Pacific MPS Registry (APMR), an electronic remote data system, was established by the APMN. Then, the Association for Research of MPS & Rare Diseases (ARMRD), an academic society that supports research on MPS and other rare diseases, was established by President Dong-Kyu Jin in April in 2015. The main task of the ARMRD is to support APMN-related work. The ARMRD published a uniform guideline that reflects the characteristics and circumstances of local patients through the Korean MPS Expert Council. Now, the APMN, APMR, and the annual Korean MPS Symposium are supported by ARMRD. Organizations like the APMN and APMR are necessary because international cooperation and collaboration are needed to conduct clinical trials on those diseases. ARMRD members hope to encourage the interest of experts and researchers of MPS & rare diseases as well as active participation in the research and treatment of patients suffering from rare diseases, including MPS, to ultimately improve the quality of life of the patients as well as their families.

• 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.

• 대한유전성대사질환학회지
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• 제10권1호
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• pp.59-66
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• 2010

Enzyme replacement of therapy (ERT) is one of the most promising therapeutic strategies for the treatment of lysosomal storage disorders. ERT is available in three types of Mucopolysaccharidosis (MPS): for MPS I (Aludrazyme$^{(R)}$), MPS II (Elaprase$^{(R)}$) and MPS VI (Naglazyme$^{(R)}$) patients who are over 5 years old. But recently, early diagnosis can be done by expert clinicians and even in prenatal case. We describe the case of ERT under 5 years old MPS patients. Up to June, 2010 in Samsung Medical Center, there are 6patients who were diagnosed as MPS and started ERT under 5 years old. 3 patients were MPS I, 3 patients were MPS II. 2 patient who was diagnosed as MPS I was female and others were male. Their age at diagnosis were 4 to 37month-old (4, 13, 16, 25, 27, 37 month-old) and they are now 9 to 60 month-old (9, 39, 32, 81, 60 month-old). The youngest patient was started ERT at 4 month-old and others were started at their 13 to 49 month-old (13, 29, 27, 28, 49 month-old). First manifested symptoms of patients were macrocephaly, kyphosis and coarse face appearance. Especially, in 2 of them, one was MPS I and the other was MPS II had elder brother with same disease. And the youngest one was diagnosed by the iduronate-2-sulfatase (IDS) gene analysis from chorionic villi sampling. His mother knew that she was a heterozygous carrier of IDS gene mutation because her younger brother died from MPS II. All of them confirmed as MPS by the enzyme assay in leukocytes and fibroblast skin culture. We started ERT with ${\alpha}$-L-iduronidase(Aldurazyme$^{(R)}$) to MPS I and did recombinant human iduronate-2-sulfatase (Elaprase$^{(R)}$) to MPS II patients as recommended dose as over 5 years old. But for MPS II patient who was 4 month old, we started ERT by recombinant human IDS (Elaprase$^{(R)}$) with reduced dose 0.1 mg/kg and increased dose every 2 weeks by 0.1mg/kg up to 0.5mg/kg IV infusion. During ERT, all patients had no adverse effects and the excretion of GAGs were decreased. We have evaluated other clinical symptoms such as liver/ spleen volume, heart function and neurologic evaluation. We describe a successful ERT to MPS I and MPS II patient under 5 years old without any adverse event. It indicates that ERT in young children are well tolerated and that it has several effects which may confer clinical benefits with long-term therapy.

• 통합자연과학논문집
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• 제9권2호
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• pp.113-120
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• 2016

Monopolar spindle 1 (Mps1) is an attractive cancer target due to its high expression levels in a wide range of cancer cells. Mps1 is a dual specificity kinase. It plays an essential role in mitosis. The high expression od Mps1 was observed in various grades of breast cancers. In the current study, we have developed a CoMFA model of pyridazine derivatives as Mps1 kinase inhibitors. The developed CoMFA model ($q^2=0.797$; ONC=6; $r^2=0.992$) exhibited a good predictive ability. The model was then validated by Leave out five, progressive sampling and bootstrapping and found to be robust. The analysis of the CoMFA contour maps depicted favorable and unfavorable regions to enhance the activity. Bulky positive substitution at $R^3$ position and Negative substitution in $R^1$ position is favored could increase the activity. In contrast, bulky substitution in $R^1$ position is not favored. Our results can be used in designing a potent Mps1 (TTK) inhibitor.

• 환경생물
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• 제42권1호
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• pp.1-14
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• 2024

This study evaluated the risk of single and combined exposure to microplastics in zebrafish (Danio rerio) through biomarkers, such as survival rate, excretion rate, and histological alterations of organ systems. The experimental groups were the control(Cont.), single microplastics exposure group(MPs, 1.83%/fish total weight(g)), the copper group(Cu, 21.6 ㎍ L^-1), and a group with combined exposure to MPs and copper (MPs^*Cu). The experiment was conducted with individual exposure (7 days) for MP excretion rate analysis and group exposure (14 days) for biomarker analysis. The daily excretion rate of MPs tended to decrease in a time-dependent manner. The copper concentration in the body was not significantly different between single and combined copper exposure. The degeneration of mucous cells in the skin, capillary dilation of the gill lamella, increased intestinal mucous, hepatocyte hypertrophy, and the degeneration of glomeruli and renal tubules were observed in all exposure groups. These histological alterations showed the highest tendency in the MPs^*Cu group. In this study, the changes in biomarkers were attributed to the single effect of copper or the combined effect of copper and MPs rather than being solely influenced by MPs.

• The Korean Journal of Pain
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• 제24권2호
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• pp.100-104
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• 2011

Background: Myofascial pain syndrome (MPS) is a regional musculoskeletal pain disorder that is caused by myofascial trigger points. The objective of this study was to determine the prevalence of MPS among chronic back pain patients, as well as to identify risk factors and the outcome of this disorder. Methods: This was a prospective observational study involving 126 patients who attended the Pain Management Unit for chronic back pain between 1st January 2009 and 31st December 2009. Data examined included demographic features of patients, duration of back pain, muscle(s) involved, primary diagnosis, treatment modality and response to treatment. Results: The prevalence of MPS among chronic back pain patients was 63.5% (n = 80). Secondary MPS was more common than primary MPS, making up 81.3% of the total MPS. There was an association between female gender and risk of developing MPS ($x^2$ = 5.38, P = 0.02, O.R. = 2.4). Occupation, body mass index and duration of back pain were not significantly associated with MPS occurrence. Repeated measures analysis showed significant changes (P < 0.001) in Visual Analogue Score (VAS) and Modified Oswestry Disability Score (MODS) with standard management during three consecutive visits at six-month intervals. Conclusions: MPS prevalence among chronic back pain patients was significantly high, with female gender being a significant risk factor. With proper diagnosis and expert management, MPS has a favourable outcome.

• 대한토목학회논문집
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• 제41권1호
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• pp.13-20
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• 2021

최근 우리나라는 주거 및 주차 공간의 확보를 위해 다양한 필로티 구조물이 건설되어 왔다. 하지만 이러한 필로티 구조물은 1층에 주차 공간을 확보하기 위해 벽체가 없는 돌출된 기둥의 형태로 건설되어 왔다. 이러한 형태는 지진 발생 시 일반적인 구조물에 비해 상대적으로 기둥의 파손이 쉽게 발생하며, 이러한 파손은 구조물의 붕괴로 이어질 수 있다. 따라서 본 연구에서는 MPS (Multi Performance System) 면진받침을 활용한 필로티 구조물의 안전성 확보에 대한 연구를 수행하였다. 기존에 건설되어 있는 필로티 구조물에 대해 MPS 면진받침 설치 유무에 따른 비선형 동적 해석을 수행하고 해석결과를 비교 및 분석하였다. 최종적으로 각각의 내진성능 평가를 수행하고 MPS 면진받침의 성능을 검증하였다.

• Journal of Periodontal and Implant Science
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• 제48권1호
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• pp.22-33
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• 2018

Purpose: The purpose of this study was to evaluate the efficacy of the modified plaque score (MPS) for assessing the oral hygiene status of periodontitis patients. Methods: A total of 116 patients were included in this study. After evaluation of the $L{\ddot{o}}e$ and Silness gingival index (GI), Silness and $L{\ddot{o}}e$ plaque index (PlI), O'Leary plaque control record (PCR), and MPS, patients were randomly assigned to either a conventional tooth brushing instruction (C-TBI) group (n=56) or a professional intraoral tooth brushing instruction (P-TBI) group (n=60). The MPS and clinical parameters were re-evaluated after scaling and a series of root planing. The convergent validity of MPS with the PlI and PCR was assessed. The measurement time for MPS and PCR was compared according to the proficiency of the examiner. Results: After root planing, the GI, PlI, PCR, and MPS improved from their respective baseline values in both groups. Three different plaque indices including the MPS, showed significant differences between the C-TBI group and the P-TBI group after root planing. The MPS showed significant concurrence with the PCR and PlI. The mean time for PCR measurement was $2.76{\pm}0.71$ times longer than that for MPS measurement after 2 weeks of training. Conclusions: MPS seems to be a practical plaque scoring system compared with the PlI and PCR. These findings suggest that repetitive plaque control combined with an easily applicable plaque index (MPS) may facilitate more effective oral hygiene education and improved periodontal health.