• Advances in Traditional Medicine
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• 제9권2호
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• pp.164-173
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• 2009

The anti-inflammatory effect of Spirulina fusiformis on monosodium urate crystal-induced inflammation in mice has been investigated and compared with the non-steroidal anti-inflammatory drug Indomethacin. The paw volume, lysosomal enzyme activities, lipid peroxidation, anti-oxidant status and inflammatory mediator tumour necrosis factor-$\alpha$ were studied in control and monosodium urate crystal-induced mice after oral administration of Spirulina platensis in an experimental model for gouty arthritis. In the induced mice, the levels of lysosomal enzymes, inflammatory mediator tumour necrosis factor-$\alpha$, lipid peroxidation and the paw volume increased significantly, whereas the antioxidant status decreased when compared to control mice. $\beta$-glucuronidase and lactate dehydrogenase level were also found to be increased in untreated monosodium urate crystal-incubated polymorphonuclear leucocytes. After the oral administration of Spirulina fusiformis, the physical and biochemical changes observed in monosodium urate crystal-induced animals were significantly restored to near normal levels. The results clearly indicated the anti-inflammatory role of Spirulina fusiformis, a promising drug for gouty arthritis.

• 대한임상검사과학회지
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• 제42권3호
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• pp.116-124
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• 2010

This study was aimed to clerify the cytotoxicity of some plant extracts such as Hosta longissima HONDA (HL), Hemerocallis fulva var. Kwanso REGL (HFVK), Hemerocallis fulva L (HF), Macrocapium officinale NAKAI (MO) and Mentha canadensis var. piperascens HARA (MCVP), the cultured NIH3T3 fibroblasts were treated with 25, 50, 100, 150 and $200{\mu}g/mL$ of five kinds of plant extracts for 48 hours, respectively. The cytotoxicity of plant extracts was measured by MTT and NR assays for the cell viability, and XTT assay for the cell adhesion activity. In this study, HL, MO and FHVK extracts showed the range of midtoxic-non toxic by the criteria of chemical cytotoxicity. While, the HF and MCVP extracts showed midtoxic. In the extract cytotoxicity, HL, MO and FHVK extracts showed non-toxic by the criteria of extract cytotoxicity. While, HF extract was determined as lower-toxic. In the responsive sensitivity of each plant extract on colorimetric assays, HF extract was sensitive to mitochondrial enzyme by MTT assay, lysosomal enzyme by NR assay and mitochondrial nucleus by XTT assay. While, MCVP extract was sensitive to mitochondrial enzyme by MTT assay and lysosomal enzyme by NR assay than other assays. While, HL, HFVK and MO extracts were most sensitive to NR assay. Cell culture is one of useful materials in the screening of cytotoxic and recovary effect on the putative chemical agents or plant extract. And also, colorimetric assay is regarded as very useful tools for quantitative measurement of cytotoxic effect on plant extracts in vitro.

• 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.

• Journal of mucopolysaccharidosis and rare diseases
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• 제5권1호
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• pp.12-16
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• 2021

Mucopolysaccharidosis type II (MPS II, Hunter syndrome) is an X-linked lysosomal storage disorder caused by deficiency of the enzyme iduronate-2-sulfatase, leading to the accumulation of glycosaminoglycans (GAGs), which affects multiple organs and systems. Current treatments for MPS II include enzyme replacement therapy (ERT) and hematopoietic cell transplantation (HCT) to reduce the accumulation of GAGs. HCT has the potential advantage that donor-derived enzyme-competent cells can provide a continuous secreting source of the enzyme. However, HCT as a treatment for MPS II remains controversial because its effectiveness is unclear, particularly in terms of neurological symptoms. To date, several clinical experiences with HCT in MPS II have been reported. In this paper, we review post-HCT outcomes in the previously published literature and discuss the effects of HCT on each of the clinical signs and symptoms of MPS II.

• Journal of mucopolysaccharidosis and rare diseases
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• 제5권1호
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• pp.22-28
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• 2021

Mucopolysaccharidosis type III (MPS III) or Sanfilippo disease is an orphan-inherited lysosomal storage disease. It is one of the most common MPS subtypes. The classical presentation is an infantile-onset neurodegenerative disease characterized by intellectual regression, behavioral and sleep disturbances, loss of ambulation, and early death. Unlike other MPS, no disease-modifying therapy has been approved. Here, we review the curative therapy developed for MPS III, from historically ineffective hematopoietic stem cell transplantation and substrate reduction therapy to the promising enzyme replacement therapy or adeno-associated/lentiviral vector-mediated gene therapy. Preclinical studies are presented with recent translational first-in-man trials. We also present experimental research with preclinical mRNA and gene-editing strategies. Lessons from animal studies and clinical trials have highlighted the importance of early therapy before extensive neuronal loss. Disease-modifying therapy for MPS III will likely mandate the development of new early diagnosis strategies.

• 대한한방내과학회지
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• 제19권1호
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• pp.491-504
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• 1998

Mainly side effects of antitumor chemotheraphy are fatigue, G-Ⅰ trouble(such as vomitting, nausea, diarrhea) and reduction of medullary function etc. Differentiated from syndromes in oriental medicine, above symptoms are recognized to 'Deficiency of both ki(vital energy) and blood'. And SDT(Sipjeondaebotang) has been widely used in 'Deficiency of both ki(vital energy) and blood'. Dr. Mun's SDTG(Sipjeondaebotanggamibang) consists of SDT plus several herb medicines-these have antitumor effect and reduce chemotherapheutic side effect. This experiment was undertaken to study the effects of SDTG on chemotherapheutic side effect and cytotoxicity. The results obtained in this study were as follows: Antitumor activities of the ethanol extract from SDTG(Sipjeondaebotanggamibang) and MMC(Mytomycin) on ascitis form of calcinoma in mice is a little improved. Especialy mean survival times of the group of SDTG(200mg/kg) and MMC(0.1mg/kg) is improved over 50%. When SDTG and MMC is administrated together, the weight of tumor is more decreased than MMC alone. The effect of the ethanol extract from SDTG and MMC on the lysosomal enzymes in Ehrich ascites carcinoma cell are more significantly improved than MMC alone. SDTG extract increases both NKcell conjugation and cyto-lysis against target cell. According to the above results it is recognized that SDTG increases the chemotherapheutic cytotoxicity of MMC and the activity of NKcell.

• 대한유전성대사질환학회지
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• 제14권2호
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• pp.178-181
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• 2014

헌터증후군(뮤코다당증 II형)은 글리코사미노글리칸의 분해를 촉매하는 효소인 iduronate-2-sulfatase 결핍에 의해 조직이나 기관의 세포 내 리소좀에 heparin sulfate와 dermatan sulfate 등의 전구물질이 축적되어 퇴행성 병변을 일으키는 유전 질환이다. 현재 효소보충요법을 통해 증상의 호전 및 질병의 진행을 지연시키는 치료가 가능하나, 중추신경계 증상이 발현된 경우 치료가 어려운 한계가 있어, 무엇보다 조기에 의심하고 진단하여 치료를 시작하는 것이 중요하다. 따라서 어린연령에 진단된 환아들의 임상적 특징에 대해 이해하는 것이 필요하며, 이에 저자들은 2.5세의 어린 연령에 진단된 환아를 경험하여 이를 보고하는 바이다.

• Journal of Radiation Protection and Research
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• 제18권2호
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• pp.61-69
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• 1993

우라늄 피폭후 효소활성도가 내부피폭의 biomarker로서의 가능성을 연구하기 위하여 수서생물인 잉어(Caprinus carpio)의 복강내 우라늄 투여시 간조직에서 일어나는 여러가지 효소계의 변화를 조사하였다. 이러한 효소활성도의 변화는 우라늄 내부피폭의 biomarker로서 가능성을 가지고 있으며 연구결과는 다음과 같다. 1) 6일째까지 우라늄을 주사하여 적출한 간에서의 전체단백질량은 계속 감소하고 있다. 2) 세포내의 lysosome내에 함유하고 있는 acid pretense와 ${\beta}-glucuronidase$의 활성도는 6일째 투여후까지 활성도가 감소하고 있다. 3) Alkaline phosphatase의 경우 6일째 우라늄 투석후까지 증가하고 있으며 반대로 acid phosphatase의 경우 6일째 우라늄 투여후까지 활성도가 급격히 증가하였고 glutamate oxaloacetate transaminase의 활성도는 완만하게 증가하고 있다. 4) Creatine kinase의 활성도는 완만한 감소를 보이고 있으며 malate dehydrogenase는 첫번 우라늄 투여후에 활성도가 급격히 감소하였고 3일째 우라늄 투여후에는 활성도가 거의 나타나지 않았다.

• Preventive Nutrition and Food Science
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• 제2권1호
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• pp.55-60
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• 1997

A lysosomal and matrix degrading enzyme $\beta$-glucuronidase activity was measured in BALS/c mice fed high and low Ca in combination with the i.p. adminstration of calcium-regulating hormones including parathyoid hormone(PTH), calcitonin(CT) and cholecalciferol(Vit D). After feeding experimental diets for five weeks, mice were sacrificed by cervical dislocation and the enzyme was fluometrically measured at 440nm. $\beta$-Glucuronidase activity was inhibited by high calcium in the diet. in addition, vitamin D also inhibited the enzyme activity in the serum regardless of the level of dietary calcium. In contrast, PTH has shown to stimulate the enzyme at all the levels of dietary calcium. Calcitonin, and inhibitor of PTH action for bone resorption, revealed to curb PTH effect in this enzyme, whereas CT stimulated the action of vitamin D in the serum. The above results led us to conclude that osteoclastic bone resorption and senile osteoporosis may be reduced by adequate dietary calcium and vitamin D.

• 대한유전성대사질환학회지
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• 제5권1호
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• pp.18-22
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• 2005

저자들은 Pompe 병으로 진단된 3세 남아에 recombinant human GAA 정주를 통한 효소 보충 치료를 시행하여 운동 능력과 심기능이 호전되며 간비대도 호전된 1례를 경험하였기에 보고하는 바이다.