• BMB Reports
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• v.51 no.1
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• pp.5-13
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• 2018

Formation of toxic protein aggregates is a common feature and mainly contributes to the pathogenesis of neurodegenerative diseases (NDDs), which include amyotrophic lateral sclerosis (ALS), Alzheimer's, Parkinson's, Huntington's, and prion diseases. The transglutaminase 2 (TG2) gene encodes a multifunctional enzyme, displaying four types of activity, such as transamidation, GTPase, protein disulfide isomerase, and protein kinase activities. Many studies demonstrated that the calcium-dependent transamidation activity of TG2 affects the formation of insoluble and toxic amyloid aggregates that mainly consisted of NDD-related proteins. So far, many important and NDD-related substrates of TG2 have been identified, including $amlyoid-{\beta}$, tau, ${\alpha}-synuclein$, mutant huntingtin, and ALS-linked trans-activation response (TAR) DNA-binding protein 43. Recently, the formation of toxic inclusions mediated by several TG2 substrates were efficiently inhibited by TG2 inhibitors. Therefore, the development of highly specific TG2 inhibitors would be an important tool in alleviating the progression of TG2-related brain disorders. In this review, the authors discuss recent advances in TG2 biochemistry, several mechanisms of molecular regulation and pleotropic signaling functions, and the presumed role of TG2 in the progression of many NDDs.

• Journal of Sasang Constitutional Medicine
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• v.18 no.2
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• pp.139-147
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• 2006

1. Objectives Creutzfelt Jacob Disease is one of a group of neurodegenerative disorders causing spongiform encephalopathies due to a infection of prion or unconventional slow virus on central nerve system. The diagnosis of this disease is not easy and there is currently no cure. This article is to report our case about a female patient who was not diagnosed as CJD at the early period so that we treated her with Yangkyuksanhwa-tang(凉膈散火湯) and Jihwangbaekho-tang(地黃白虎湯). 2. Methods Magnetic resonance imaging(brain MRI), blood test and computer tomography were performed. The treatment for this patient was clinically based on Sasang Constitutional Medicine. 3. Results and Conclusions (1) Visual field defect, ataxia, myoclonus, sweating and dysuria were the main symptoms of the patient. (2) The pathological change in parenchyme was not revealed during the early periods by MR imaging. So the diagnosing CJD was not possible during the time in this case. (3) Jihwangbaekho-tang(地黃白虎湯) improved her myoclonus and sweating. Bur her mental disorder and the progress of the pathological change in the parenchyme was not able to be treated.

• 한국생물공학회:학술대회논문집
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• 2005.04a
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• pp.203-206
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• 2005

TGE (Transmissible Gastroenteritis) caused by a virus belonging to family coronavirus, results in an acute infection of the small intestine of the pig. The optimum operation variables such as multiplicity of infection (MOI), infection time and harvest time were investigated for TGE vaccine production by immobilized ST(swine testicle) cells. In the culture supplemented with 5% serum, maximum virus titer of $1.2{\times}10^6pfu/ml$ was obtained at the conditions of 0.01 MOI, 2day infection time, and 1 day harvest time. Serum is a potential source of bacterial, mycoplasmal and viral contamination, and it has a possibility of the introduction of serum proteins, prion and pyrogens into the final product. For these reasons, much attention has been focused on the development of serum-free media. A new serum-free media (SFM) has been developed in order to produce TGE vaccine of high quality with low cost. The performance of SFM developed was compared with other commercially available serum-free media and serum supplemented media in terms of virus productivity. The cultures with serum-free media showed higher titer than that with serum supplemented media. Among various serum-free media tested, CHO-S-SFMII showed highest virus titer.

• IMMUNE NETWORK
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• v.13 no.4
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• pp.148-156
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• 2013

The $PrP^C$ is expressed in many types of immune cells including monocytes and macrophages, however, its function in immune regulation remains to be elucidated. In the present study, we examined a role for $PrP^C$ in regulation of monocyte function. Specifically, the effect of a soluble form of $PrP^C$ was studied in human monocytes. A recombinant fusion protein of soluble human $PrP^C$ fused with the Fc portion of human IgG1 (designated as soluble $PrP^C$-Fc) bound to the cell surface of monocytes, induced differentiation to macrophage-like cells, and enhanced adherence and phagocytic activity. In addition, soluble $PrP^C$-Fc stimulated monocytes to produce pro-inflammatory cytokines such as $TNF-{\alpha}$, $IL-1{\beta}$, and IL-6. Both ERK and $NF-{\kappa}B$ signaling pathways were activated in soluble $PrP^C$-treated monocytes, and inhibitors of either pathway abrogated monocyte adherence and cytokine production. Taken together, we conclude that soluble $PrP^C$-Fc enhanced adherence, phagocytosis, and cytokine production of monocytes via activation of the ERK and $NF-{\kappa}B$ signaling pathways.

• Asian-Australasian Journal of Animal Sciences
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• v.20 no.5
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• pp.653-660
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• 2007

In this study, we have investigated sequence variants in the PRNP gene of 20 individuals belonging to the Korean cattle, and have analyzed and compared genetic features between varieties of other cattle breeds. Of the 73 sequence variants identified in Korean cattle, 27 were identified for the first time in this study, whereas 46 of these polymorphisms had previously been isolated. We discovered a 2.6 kb SNP hot spot region localized on the putative promoter region of the PRNP gene. Furthermore, the copy numbers of the octapeptide repeat (24 bp indel) which is detected on the coding sequence (CDS) of the PRNP exhibited a completely homozygous 6/6 genotype which is dominant in other cattle breeds. We also characterized a new 19 bp/10 bp allele located on the putative promoter region of the PRNP gene, which represented 0.71 in allele frequency. To the best of our knowledge, this report is the first to address polymorphisms of the PRNP gene structure in Korean cattle in which BSE has yet to be discovered. Therefore, our findings may prove useful with regard to our current understanding of allelic diversity in bovine species, and may also provide new insights into the genetic factors associated with susceptibility or resistance to BSE.

• Korean journal of applied entomology
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• v.33 no.2
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• pp.60-65
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• 1994

This study was carried out to investigate the ecology of Nesodiprion japonlcus (Mmm,m) damaging many conifers The results obtained were as follows: Nesod~prion japonicus (Maun) emerged two times for a year and their body was black m both sexes. A female (10.1t 1 2 mm) adult was larger than a male (9.9t 0.8 mm) one in the body size. The size of antenna as plumose of female was different from that of male. Life span of adults was 3 or 4 days, those emergence rate was 92% and sex ratio of female to male was 6: 4. Adults 1a1d only an egg in the leaf and the region of the leaf aviposited turned brown. Duration of egg was 14.35 1 2 days, egg was In the shape of banana and red~sh-yellow,a nd its sire was 2.4+ 0.2 mm long. Duration of larvae was 30.35 2.1 days and its slze 20.15 2.2 mm, body was green with grey~sh shipes on the dorsal and lateral aspect, the tip of front and hind was yellow and head was brown with a dark-p~gmented area. Mature larva turned into light green and get lost a dark-pigmented area of head. The firstly-emerged larva made cocoons on the base of leaves and the secondawemerged larva among fallen leaves Duration of prepupa was 14.3f 2.0 days and their size 10.15 2.3 mm Duration of pupa was 12.2+ 1.3 days and its size 10.lt0.2mm.

• Korean Journal of Veterinary Service
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• v.32 no.4
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• pp.307-314
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• 2009

The aim of the present study was to investigate whether Bovine Spongiform Encephalopathy (BSE) is present in this country and to analyze the Global BSE Risk (GBR) status in Bangladesh. A total of 2,000 brain samples were collected from cattle older than 30 months of age, slaughtered for human consumption in the district slaughter houses from 2005 to 2006. The brainstem (obex), Pyriform lobe, cerebrum and cerebellum were subjected to histopathological study. Samples that showed some nonspecific lesions were subjected to immunohistochemistry and only brain stem to ELISA for the detection of abnormal prion protein $PrP^{sc}$. In passive surveillance, annual overall diseases of cattle, buffalo, sheep and goats in Bangladesh were collected from Department of Livestock Services (DLS), Dhaka to investigate the occurrences of neurological diseases. Import related data were collected from "National Export Promotion Bureau" Kawran Bazar, Bangladesh Bank and DLS to analyze the importing products of animal origin (cattle, buffalo, sheep and goats) from different countries to find whether or not the imported products posed any risk for the BSE. In an actire surveillance conducted in slaughter house, histopathologically BSE specific lesions were not detected in any of the brain samples, but other nonspecific lesions were observed. No $PrP^{sc}$ was detected from the samples by immunohistochemistry and ELISA. DLS report also supported the absence of BSE in cattle and buffalo and scrapie in sheep and goats in Bangladesh. It was also clearly recorded that Bangladesh imported livestock products from countries in GBR level I and II but not from countries in GBR level III and IV. From this study it apparently seems that BSE is not currently present in the indigenous animals in Bangladesh and poses no or negligible risk to human and animal health.

• Journal of Life Science
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• v.17 no.8 s.88
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• pp.1129-1134
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• 2007

In the previous our study, a cDNA that encodes protein disulfide isomerase from Bombyx mori (bPDI)was isolated and characterized. bPDI has an open reading frame of 494 amino acids contained two PDI-typical thioredoxin active site of WCGHCK and ER (endoplasmic reticulum) retention signal of the KDEL motif at its C-terminal. Recent studies have demonstrated that misfolded proteins are accumulated in many diseases including Alzheimer’s, goiter, emphysema, and prion infections. bPDI was over-expressed or knock-downed in Sf9 cells to study the relationship between bPDI expression and protections against protein misfolding. bPDI gene was cloned in insect expression vector pIZT/V5-His for over-expression and bPDI double-stranded RNA (dsRNA) was generated for knock-down. Over-expression of bPDI significantly improved survival rate, but bPDI dsRNA transfection significantly reduced survival rate after 48 hours exposure. In mock-transfected or wild-type cells had no significant effect. The results support the view that bPDI is one of the important intracellular components for cell protect mechanism, especially, against ER stress such as protein misfolding.