• Journal of the Korean Society of Tobacco Science
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• v.27 no.2
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• pp.212-218
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• 2005

Cigarette smoking has been known to have a few beneficial effects on some neuronal diseases such as Alzheimer's disease(AD), Parkinson's disease(PD) and prion disease by scrapie agent shows many similar properties with AD. In this respect, we investigated what biological effects are exerted by cigarette smoke exposure(CSE) in the brain of mouse infected by 87V scrapie. The scrapie agent was inoculated through stereotaxic microinjection of the homogenates of the scrapie agent infected brain into the intracerebral system in the 1M mice. The inoculation into mice typically exhibits neurochemical, physiological and histopathological characteristics of prion disease: loss of neurotransmitters and induction of astrocytosis and vacuolation in brain as well as reduction of spatial movement and loss of body weight. CSE led to alleviated the loss of body weight and also improved spatial movement of the infected mice. Most interestingly, CSE attenuated astrocytosis and vacuolation caused by scrapie infection in the brain. In addition, decreased levels of dopamine in striatal and hypothalamic regions as well as serotonin level in hippocampus caused by scrapie infection were also attenuated by exposure to cigarette smoke. These findings suggest that cigarette smoke, by its inhibition of astrocytosis and vacuolation followed by its restoration of levels of some neurotransmitters, may partly contribute to suppression in the progress of neurodegeneration caused by scrapie infection.

• The Journal of Korean Society of Virology
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• v.28 no.2
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• pp.183-192
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• 1998

DNA methylation degree in the several murine brain and liver genes of different ages and after scrapie infection have been examined by using methylation-sensitive restriction endonuclease digestion. We found that the methylation of c-fos and c-myc in the brain and liver was increased during the late fetal to one month postnatal developmental periods. However, those of the SGP-2, $S100{\beta}$, APP950, PrP, and APLP1 genes were decreased at the same periods. The comparison of the DNA methylation patterns between scrapie infected brains and controls demonstrated there is no significant difference in methylation degree of scrapie-infected brains. These observations indicate that DNA methylation might be importantly related to the aging process. The scrapie-infected murine brain was not significantly developed more senescent than the same age-controls did.

• Journal of the Korean Society of Tobacco Science
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• v.31 no.1
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• pp.29-38
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• 2009

Although prion diseases, a group of fatal neurodegenerative diseases of human and animals, are presumed to be caused by several mechanisms including abnormal change of prion protein, oxidative stress is still believed to play a central role in development of the diseases. Cigarette smoking has a few beneficial effects on neuronal diseases such as Alzheimer's disease and Parkinson's disease despite of many detrimental effects. In this study, we investigated how chronic cigarette smoking could exert such beneficial effect against oxidative damage. For this study, homogenates of 87V scrapie-infected brain was inoculated on intracerebral system of IM mice through stereotaxic microinjection and biochemical properties concerning with oxidative stress were examined. The scrapie infection decreased the activity of mitochondrial Mn-containing superoxide dismutase by 50% of the control, meanwhile the effects on other antioxidant enzymes including Cu or Zn-containing superoxide dismutase were not significant. Additionally, the infection elevated superoxide level as well as monoamine oxide-B (MAO-B) in the infected brain. Interestingly, many of the detrimental effects were improved in partial or significantly by long-term cigarette smoke exposure (CSE). CSE not only completely prevented the generation of mitochondrial superoxide but also significantly (p<0.05) decreased the elevated mitochondrial MAO-B activity in the infected brain. Concomitantly, CSE prevented subsequent protein oxidation and lipid peroxidation caused by scrapie infection; however, it did not affect the activities of antioxidant enzymes. These results suggest that chronic exposure of cigarette smoke contribute to in part preventing the progress of neurodegeneration caused by scrapie infection.

• Asian-Australasian Journal of Animal Sciences
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• v.21 no.7
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• pp.936-940
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• 2008

The PCR-amplified prion protein (PrP) gene was sequenced to determine the frequency of scrapie-associated as well as novel PrP genotypes in 72 healthy goats representing five breeds. A total of six genotypes were detected, resulting from the three reported 143 (H/R), 154 (R/H) and 240 (S/P) and the two novel 39 (S/R) and 185 (I/F) amino acid polymorphisms. Of the four silent mutations 42 (a$\rightarrow$g), 138 (c$\rightarrow$t), 231 (c$\rightarrow$a) and 237 (g$\rightarrow$c) detected in this study, 237 (g$\rightarrow$c) is novel. A genotype (SIP/RFP) harboring three amino acid polymorphisms 39 (S/R), 185 (I/F) and 240 (S/P) was found in few goats. Although both scrapie-associated genotypes with 143 (H/R) and 154 (R/H) polymorphisms and others with 39 (S/R), 185 (I/F) and 240 (S/P) polymorphisms were present in the studied Pakistani goats, their frequency was lower than that of the wild-type genotype SHRIS/SHRIS (34.7%). These results emphasize the need for further sequencing of the PrP gene in a large number of goats representing the five studied breeds, so that overall PrP variability can be assessed in these breeds in research addressing future concerns about scrapie.

• Journal of Microbiology and Biotechnology
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• v.26 no.9
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• pp.1657-1660
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• 2016

Prion diseases are incurable neurodegenerative disorders. Our previous study demonstrated that polylysine was effective in prolonging the incubation period in a rodent model and in alleviating the scrapie prion protein (PrP^Sc) burden in the brain at the terminal stage of the disease. Here, we report that intraperitoneal administration of polylysine suppresses the accumulation of prions in the spleen during the early stages of the disease. This study supports the congruence of PrP^Sc inhibition by polylysine in both the spleen and brain.

• Microbiology and Biotechnology Letters
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• v.43 no.2
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• pp.91-96
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• 2015

Prions are proteinaceous infectious particles that cause neurodegenerative diseases, such as scrapie in sheep, bovine spongiform encephalopathy in cattle and Creutzfeldt-Jakob disease (CJD) in humans. Although the detailed process, regarding the abnormal conversion of prion proteins (PrP), remains to be fully elucidated, a number of environmental factors appear to affect the formation of misfolded PrP, termed PrP^Sc. Because oceanic algae contain mycosporine-like amino acids (MAAs), which exhibit cellular defensive activities under a variety of stress conditions, we investigated the level of PrP^Sc in prion-infected neuroblastoma cells using mycosporine-glycine, porphyra-334 and shinorine. When judged by the level of protease-resistant PrP^Sc in western blots, porphyra-334 and shinorine increased the level of PrP^Sc in cells, but mycosporine-glycine did not. The current results indicate that the MAAs tested in this study enhance the formation of PrP^Sc.

• Development and Reproduction
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• v.28 no.2
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• pp.29-36
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• 2024

Cellular prion protein (PrP^C) encoded at Prnp gene is well-known to form a misfolded isoform, termed scrapie PrP (PrP^SC) that cause transmissible degenerative diseases in central nervous system. The physiological role of PrP^C has been proposed by many studies, showing that PrP^C interacts with various intracellular, membrane, and extracellular molecules including mitochondrial inner membrane as a scaffold. PrP^C is expressed in most cell types including reproductive organs. Numerous studies using PrP^C knockout rodent models found no obvious phenotypic changes, in particular the clear phenotypes in development and reproduction have not demonstrated in these knockout models. However, various roles of PrP^C have been evaluated at the cellular levels. In this review, we summarized the known roles of PrP^C in various cell types and tissues with a special emphasis on those involved in reproduction.

• Journal of the korean veterinary medical association
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• v.32 no.4
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• pp.234-246
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• 1996

양의 스크래피(scrapie)는 우리나라에서의 발생보고가 없는 해외가축전염병의 일종이며, 동물의 전염성 해면형 뇌증(Transmissible Spomgifrom Encephalopathies; TSEs)중 역사가 가장 오랜 질병인데, 현재 영국에서 문제되고 있는 새로운 전염병인 소 해면형 뇌증(Bovine Spongiform Encephalopathy; BSE) 즉 일명 광우병(mad cow disease)과의 어떤 연관 가능성 때문에 수의학계의 관심의 대상이 되고 있는 질병이다. 일단 감염되어 발병되면 치료대책 없이 100$\%$ 폐사되는 세기의 불치병으로 알려진, 소 해면형 뇌증(BSE)은 영국에서 현재 사람의 크로이츠휄트-야콥병(Creutzfeldt-Jakob disease; CJD)과의 관련 가능성 여부를 놓고 독특한 문제가 되고 있는 세계적인 희귀질병이다. 이들 질병에 대하여는 아직까지 확실한 병인체가 밝혀져 있지도 않으며, 그렇기 때문에 면역 혈청학적 진단방법도 확립되어 있지 못할 뿐만아니라 예방백신의 개발 또한 불가능하다. 다만, 임상적인 병력과 임상소견, 뇌조직 표본에 대한 현미경 검사 또는 전자현미경 검사에 의한 특이소견 관찰 등 조직병리학적 진단만이 가능할 뿐이다. 본편에서는 소 해면형 뇌증(BSE)의 병리학적 감별진단과 관련, 지금까지 보고된 임상증상을 검토해보고, 우리나라에서 경험한 소의 광견병에 대한 조직병리학적 진단 재료를 근거로하여 감별진단을 위한 참고자료로 설명하고, 국제수역사무국(Office de International Epizooties; O.I.E.)에서 발생한 Manual of Standards for Diagnostic Tests and Vaccines for List A and B Diseases of Mammals, Birds and Bees(1992)(포유류, 조류, 꿀벌에 있어서의 A급 및 B급 질병에 대한 진단시약 및 예방백신에 대한 표준지침) 중에서 소 해면형 뇌증(B 83; p 742-747)과 스크래피(B 32; p 424-427)에 관한 내용 (Chapter 22, 205-215)을 기본자료로 제공하고자 한다.

• Asian-Australasian Journal of Animal Sciences
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• v.21 no.7
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• pp.941-946
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• 2008

Single-minded 1 gene (SIM1) is a homolog of Drosophila SIM1 gene which plays a key role in the midline cell lineage of the central nervous system and is implicated in the regulation of feeding behavior and obesity in the human and mouse. In this study, porcine SIM1 gene was firstly mapped to chromosome 1p13 using radiation hybrid (RH) mapping and two polymorphisms were detected at position 607 (A/G) in SIM1 intron7 and position 780 (C/T) in SIM1 exon8. The last substitution was genotyped in a 364 F2 animal-population and an association analysis of these genotypes was performed with growth, carcass and meat quality traits by the statistical animal model. The results showed the significant influence of the SIM1 genotype on growth (p<0.05): live weight at birth, later period of growth and average daily gain; and effects on carcass composition (p<0.05): weight of head and buck kneed foreleg, backfat depth, loin eye area, carcass leaf fat and ham fat weights; and traits related to intramuscular fat content (p<0.05).

• Journal of the Korean Magnetic Resonance Society
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• v.15 no.2
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• pp.175-186
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• 2011

Amyloid fibrils have long been known to be the well known ${\alpha}$-helix to ${\beta}$-sheet transition characterizing the conversion of cellular to scrapie forms of the prion protein. A very short sequence of Yeast prion-like protein, GNNQQNY (SupN), is responsible for aggregation that induces diseases. KSI-fused tandem repeats of SupN vector are constructed and used to express SupN peptide in Escherichia coli (E.Coli). A method for a production, purification, and cleavage of tandem repeats of recombinant isotopically enriched SupN in E. coli is described. This method yields as much as 20 mg/L of isotope-enriched fusion proteins in minimal media. Synthetic SupN peptides and $^{13}C$ Gly labeled SupN peptides are studied by Congo Red staining, Birefringence and transmission electron microscopy to characterize amyloid fibril formation. To get a better understanding of aggregation-structure relationship of 7 residues of Yeast prion-like protein, the change of a conformational structure will be studied by $^{13}C$ solid-state nmr spectroscopy as powder of both amorphous and fibrillar forms.