• Journal of Interdisciplinary Genomics
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• v.1 no.1
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• pp.6-9
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• 2019

In this report, the phenotypes of three patients from two families with mucopolysaccharidosis type II (MPS II) are compared: a novel variant and recombinant variant of IDS gene. The results of urine in patients showed a pronounced increase in glycosaminoglycan excretion with decreased iduronate-2-sulfatase enzyme activity in leukocyte, leading to a diagnosis of MPS II. A patient has a novel variant with 1 bp small insertion, c.1224_1225insC in exon 9, which caused frameshifts with a premature stop codon, and two patients have a recombination variant, c.418+495_1006+1304del, leading to the loss of exons 4, 5, 6, and 7 in genomic DNA, which is relatively common in Korean patients. They had different phenotypes even in the same mutation. The patients have now been enzyme replacement therapy with a significant decrease in glycosaminoglycan excretion. Further study on residual enzyme activity, as well as experience with more cases, may shed light on the relationship between phenotypes in MPS II and gene mutations.

• Toxicological Research
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• v.34 no.4
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• pp.373-373
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• 2018

• Proceedings of the PSK Conference
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• 2003.04a
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• pp.211.2-211.2
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• 2003

Glycosaminolycans (GAGs). such as heparin and heparan sulfate, are highly charged molecules and are of great biological importance. Protein-GAGs interactions play prominent roles in cell-cell recognition and cell growth. Acharan sulfate (AS), isolated from the giant African snail Achatina fulica, is a novel member of glycosaminoglycan families. It showed antitumor activity by the inhibition of angiogenesis. (omitted)

• Journal of Physiology & Pathology in Korean Medicine
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• v.31 no.1
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• pp.46-51
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• 2017

The aim of this study is to investigate anti-arthritis activity using a KV pharmacopuncture. KV pharmacopuncture was inserted at BL40 for 4 weeks to SD-Rat, where arthritis was induced by monosodium iodoacetate (MIA) at 60 mg/ml. MMP-9, CTX II, LTB4, calcitonin and glycosaminoglycan level in serum were measured by ELISA. The cartilage of patella volume was examined and 3-D high-resolution reconstructions of the cartilage of patella were obtained using a Micro-CT system. Also, The histopathological change of knee was observed by H&E and safranin-O staining. Production of MMP-9, CTX II and LTB4 level in serum was decreased, respectively, in comparison with control. The other way, production of calcitonin and glycosaminoglycan level in serum was increased, respectively, in comparison with control. The cartilage of patella volume increased significantly. In addition, the KV group showed a increase in the cartilage volume and proteoglycan. These results may be used a remedy for new korea medicine to ease the symptoms mentioned above.

• Journal of Korean Neurosurgical Society
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• v.43 no.3
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• pp.149-154
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• 2008

Objective: The aim of this study is to elucidate the effects of transforming growth factor-${\beta}$ (TGF-${\beta}$)1 and L-ascorbic acid on proteoglycan synthesis, and the relationship between Sox9, proteoglycan, and TGF-${\beta}1$ in intervertebral disc cells. Methods: Human intervertebral disc tissue was sequentially digested to 0.2% pronase and 0.025% collagenase in DMEM/F-12 media and extracted cells were cultured in $37^{\circ}C$, 5% $CO_2$ incubator. When intervertebral disc cells were cultured with TGF-${\beta}1$ or L-ascorbic acid, the production level of sulfated glycosaminoglycan (sGAG) was estimated by dimethyl methyleneblue (DMMB) assay. The changes of Sox9 mRNA and protein levels via TGF-${\beta}1$ were detected by RT-PCR and Western blot analysis in each. Results: The amount of sGAG was increased with the lapse of time during incubation, and sGAG content of pellet cultured cells was much larger than monolayer culture. When primary cultured intervertebral disc cells in monolayer and pellet cultures were treated by TGF-${\beta}1$ 20 ng, sGAG content of experimental group was increased significantly compared to control group in both cultures. L-Ascorbic acid of serial concentrations (50-300 ug/ml) increased sGAG content of mono layer cultured intervertebral disc cells significantly in statistics. The co-treatment of TGF-${\beta}1$ and L-ascorbic acid increased more sGAG production than respective treatment. After treating with TGF-${\beta}1$, Sox9 mRNA and protein expression rates were significantly increased in disc cells compared with the control group. Conclusion: This study suggests that TGF-${\beta}1$ would increase sulfated glycosaminoglycan (sGAG) and other proteoglycans such as versican by elevating Sox9 mRNA and protein expressions in order.

• The Journal of Korean Medicine
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• v.28 no.4
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• pp.148-157
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• 2007

Background & Objective: Articular cartilage is a potential target for drugs designed to inhibit the activity of matrix metalloproteinases (MMPs) to stop or slow the destruction of the proteoglycanand collagen in the cartilage extracellular matrix. The purpose of this study was to investigate the effects of Cinnamomum cassia in inhibiting the release of glycosaminoglycan (GAG), the degradation of collagen, and MMP activity in rabbit and human articular cartilage explants. Methods: The cartilage-protective effects of Cinnamomum cassia were evaluated by using glycosaminoglycan degradation assay, collagen degradation assay, colorimetric analysis of MMP activity, measurement of lactate dehydrogenase activity and histological analysis in rabbit cartilage explants culture. Results: Interleukin-1a (IL-1a) rapidly induced GAG, but collagen was much less readily released from cartilage explants. Cinnamomum cassia significantly inhibited GAG and collagen release in a concentration-dependent manner. Cinnamomum cassia dose-dependently inhibited MMP-1, MMP-3 and MMP-13 activities from IL-1a-treated cartilage explants culture when tested at concentrations ranging from 0.02 to 1 mg/ml. Conclusion : These results indicate that Cinnamomum cassia inhibits the degradation of proteoglycan and collagen through the down regulation of MMP-1, MMP-3 and MMP-13 activities of IL-1a-stimulated rabbit and human articular cartilage explants.

• The Journal of the Korean dental association
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• v.53 no.12
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• pp.935-948
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• 2015

목적: 법랑기질 유도체(EMD)가 사람 치주인대 줄기세포(hPDLSC)의 조직 형성능에 미치는 영향을 in vitro와 in vivo 분석 모델을 이용해 평가한다. 재료 및 방법: hPDLSC를 배양하여 운반체와 함께 면역 억제된 쥐 등에 이식하였다; (1)대조군: EMD 처치하지 않은 운반체에 심어진 hPDLSC군 ($EMD^-/hPDLSC^+$), (2)실험군: EMD 처치한 운반체에 심어진 hPDLSC군 ($EMD^+/hPDLSC^+$). 각 군당 5마리씩 시행하고 8주 후 희생하였다. 조직학적, 조직계측학적 분석을 통해 형성된 백악질의 면적과 백악세포의 수 그리고 샤피 섬유의 수를 계측하였으며 면역조직화학적 분석을 통해 백악질과 교원질 형성을 평가하였다. 또한 in vitro에서 hPDLSC의 수용성 교원질과 glycosaminoglycan 형성에 대한 EMD의 효과를 분석하였다. 결과: 조직학적 분석에서 교원질성 치주 인대 조직이 실험군에서 현저하게 많이 생성된 것을 관찰할 수 있었다. 형성된 백악질의 면적과 백악세포의 수는 군 간 차이가 없었으나, 새롭게 형성된 샤피 섬유의 수는 실험군에서 대조군보다 유의하게 많았다(p<0.05). 교원질 형성에 대한 면역조직 화학적 분석 결과, 실험군에서 I, III형 교원질과 hydroxyproline의 발현이 높았다. 또한 in vitro에서 hPDLSC에 의한 수용성 교원질과 glycosaminoglycan 형성이 EMD의 농도에 비례하여 증가하였다 (p<0.05). 결론: EMD는 hPDLSC에 의한 샤피 섬유 및 교원질 생성을 증가시키고, 이는 새로운 백악질의 기능적 부착과 치주조직 재생에 중요한 역할을 한다.

• Journal of Acupuncture Research
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• v.22 no.2
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• pp.191-201
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• 2005

Background & Objective: Articular cartilage is a potential target for drugs designed to inhibit the activity of matrix metalloproteinases (MMPs) to stop or slow the destruction of the proteoglycan and collagen in the cartilage extracellular matrix. The purpose of this study was to investigate the effects of Aralia cordata Thunb. in inhibiting the release of glycosaminoglycan (GAG), the degradation of collagen, and MMP activity in rabbit articular cartilage explants. Methods : The cartilage-protective effects of Aralia cordata Thunb. were evaluated by using glycosaminoglycan degradation assay, collagen degradation assay, colorimetric analysis of MMP activity, measurement of lactate dehydrogenase activity and histological analysis in rabbit cartilage explants culture. Results : Interleukin-la (IL-1a) rapidly induced GAG, but collagen was much less readily released from cartilage explants. Aralia cordata Thunb. significantly inhibited GAG and collagen release in a concentration-dependent manner. Aralia cordata Thunb. dose-dependently inhibited MMP-3 and MMP-13 expression and activities from IL-1a-treated cartilage explants cultures when tested at concentrations ranging from 0.02 to 0.2 mg/ml. Aralia cordata Thunb. had no harmful effect on chondrocytes viability or cartilage morphology in cartilage explants. Histological analysis indicated that Aralia cordata Thunb. reduced the degradation of the cartilage matrix compared with that of IL -1a-treated cartilage explants.

• The Korea Journal of Herbology
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• v.32 no.2
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• pp.41-47
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• 2017

Objective : The aim of this study is to investigate anti-arthritis activity using a korea's natural monument No. 265 designate 'Yeonsan-Ogye'. In this study, research by using extracts from different concentration of the Yeonsan-Ogye through an MIA-induced arthritis animal model was being conducted in vivo and scientifically verifying the efficacy of medicinal food. Methods : Yeonsan-Ogye was administered 500 mg/kg/day, 1000 mg/kg/day, 2000 mg/kg/day to SD-Rat, where arthritis was induced by monosodium iodoacetate (MIA) at $60mg/m{\ell}$. MMP-9, COMP, CTXII, calcitonin and glycosaminoglycan level in serum were measured by ELISA. The changes of relative hind paw weight bearing ratio by Incapacitance Test Meter and The cartilage of meniscus volume was examined and 3-D high-resolution reconstructions of the cartilage of meniscus were obtained using a Micro-CT system. Also, the histopathological analysis of knee was observed by H&E and safranin-O staining. Results : Production of MMP-9, COMP (all groups) and CTXII (500, 1000 mg/kg/day) level in serum was decreased, respectively, in comparison with control. The other way, production of calcitonin (500, 1000 mg/kg/day) and glycosaminoglycan (all groups) level in serum, Hind paw weight bearing ratio (all groups) was increased, espectively, in comparison with control. The cartilage of patella volume in micro-CT increased significantly. In addition, all groups showed a increase in the cartilage volume and proteoglycan. Conclusion : The results for Yeonsan-Ogye showed significant antiarthritis activity in serum and the cartilage. Therefore, it is thought to be that Yeonsan Ogye can be utilized as a variety of new korea medicie and health foods against arthritis diseases.

• The Korea Journal of Herbology
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• v.32 no.6
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• pp.63-69
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• 2017

Objectives : The purpose of this study is to investigate the preventive and therapeutic efficacy of osteoarthritis using a Yeonsan-Ogye egg. so, we researched at effects of Yeonsan-Ogye egg extract on MIA-induced Osteoarthritis animal models. Methods : Yeonsan-Ogye egg extract was administered 500 mg/kg/day, 1000 mg/kg/day and 2000 mg/kg/day to SD-Rat for 2 weeks. After that, osteoarthritis was induced with $60mg/m{\ell}$ of monosodium iodoacetate (MIA) and futher administration was continued for 4 weeks. 3D imaging of cartilage patella were obtained using a Micro-CT system and the pathology change of knee was observed by H&E and safranin-O staining. The weight bearing ratio was measured by incapacitance test meter. MMP-2, MMP-9, COMP, CTX II, calcitonin and glycosaminoglycan level in serum were measured using a ELISA. Results : Micro-CT and Histopathological analysis showed the volume of the patella cartilage and the proteoglycan contents were increased in all groups. also weight bearing ratio was decreased in all groups compared with control group. Calcitonin production was increased in and 2000 mg/kg/day group and glycosaminoglycan production was increased in all groups. In addition, MMP-2, MMP-9, COMP and CTX II production were decreased in 1000 and 2000 mg/kg/day groups respectively in comparison with control. Conclusions : The results for Yeonsan-Ogye egg showed prevention and treatment efficacy against arthritis at serum and the cartilage. These results may be used a remedy for new korea medicine to ease the symptoms mentioned above. also, suggest that Yeonsan-Ogye egg can be used preventive and therapeutic material for osteoarthritis.