• Korean Journal of Radiology
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• 제2권4호
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• pp.192-196
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• 2001

Objective: To compare conventional and diffusion-weighted MR imaging in terms of their depiction of the abnormalities occurring in Creutzfeldt-Jakob disease. Materials and Methods: We retrospectively analyzed the findings of conventional (T2-weighted and fluid-attenuated inversion recovery) and diffusion-weighted MR imaging in four patients with biopsy-proven Creutzfeldt-Jakob disease. The signal intensity of the lesion was classified by visual assessment as markedly high, slightly high, or isointense, relative to normal brain parenchyma. Results: Both conventional and diffusion-weighted MR images demonstrated bilateral high signal intensity in the basal ganglia in all four patients. Cortical lesions were observed on diffusion-weighted MR images in all four, and on fluid-attenuated inversion recovery MR images in one, but in no patient on T2-weighted images. Conventional MR images showed slightly high signal intensity in all lesions, while diffusion-weighted images showed markedly high signal intensity in most. Conclusion: Diffusion-weighted MR imaging is more sensitive than its conventional counterpart in the depiction of Creutzfeldt-Jakob disease, and permits better detection of the lesion in both the cerebral cortices and basal ganglia.

• 대한신경과학회지
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• 제36권4호
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• pp.345-349
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• 2018

Familial Creutzfeldt-Jakob Disease (fCJD) is characteristic with older age onset, relatively low occurrence rate, slower progression and lower possibility of developing myoclonus, cerebellar, pyramidal signs and visual disturbance compared with classical sporadic CJD. We report a case of 75-year-old male patient presented with sudden onset of right side weakness with Broca's aphasia who has been diagnosed with fCJD with V180I mutation. This case indicates that fCJD with V180I mutation can have stroke-like initial presentation.

• 대한자기공명의과학회:학술대회논문집
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• 대한자기공명의과학회.한국자기공명학회 2005년도 공동학술대회
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• pp.73-73
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• 2005

• 대한수의사회지
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• 제32권3호
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• pp.137-142
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• 1996

최근 소의 광우병 발생보고가 영국에서 16만여두 그리고 EU 14게국에서 약 150여 마리에 이르므로써 우산업분야에 미치는 심각한 경제적 여파와 인체에서의 Creutzfeldt-Jakob병과 어떤 연관성 논란으로 공중보건상 심각한 논쟁이 일고 있다. 본회 학술홍보 위원회에서는 이 질병에 관한 현재까지의 알려진 일반적인 지식을 정리하여 회원들에게 우선 소개하고자한다. 이 질병에 관한 보다 정리된 종합적인 내용은 수의과학연구소 해외전염병과에서 정리하여 다음호에 특집으로 소개할 것입니다.

• 한국수산과학회지
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• 제47권4호
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• pp.341-346
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• 2014

Transmissible spongiform encephalopathies (TSEs), also termed prion diseases, are a threat to food safety and to human and animal health. Variant Creutzfeldt-Jakob disease (vCJD) in humans is caused by the consumption of meat contaminated with bovine spongiform encephalopathy (BSE, mad cow disease). The BSE epidemic in the United Kingdom was shown to be related with the extensive use of BSE-contaminated meat-and-bone meal (MBM) and bovine offal. Many countries worldwide use MBM, as well as meat from cows, for aquaculture feed. This raises concerns about the safety of farmed fish, a major protein source for humans. The present work reviews recent studies on fish prion protein and the transmissibility of mammalian prion agents to fish, providing insights into the future direction of fish prion research.

• Journal of Microbiology and Biotechnology
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• 제17권7호
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• pp.1059-1070
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• 2007

Prion diseases, often called transmissible spongiform encephalopathies (TSEs), are infectious diseases that accompany neurological dysfunctions in many mammalian hosts. Prion diseases include Creutzfeldt-Jakob disease (CJD) in humans, bovine spongiform encephalopathy (BSE, "mad cow disease") in cattle, scrapie in sheep, and chronic wasting disease (CWD) in deer and elks. The cause of these fatal diseases is a proteinaceous pathogen termed prion that lacks functional nucleic acids. As demonstrated in the BSE outbreak and its transmission to humans, the onset of disease is not limited to a certain species but can be transmissible from one host species to another. Such a striking nature of prions has generated huge concerns in public health and attracted serious attention in the scientific communities. To date, the potential transmission of prions to humans via foodborne infection and iatrogenic routes has not been alleviated. Rather, the possible transmission of human to human or cervids to human aggravates the terrifying situation across the globe. In this review, basic features about prion diseases including clinical and pathological characteristics, etiology, and transmission of diseases are described. Based on recently accumulated evidences, the molecular and biochemical aspects of prions, with an emphasis on the molecular interactions involved in prion conversion that is critical during prion replication and pathogenesis, are also addressed.

• EDISON SW 활용 경진대회 논문집
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• 제6회(2017년)
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• pp.32-38
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• 2017

Prion is a group of the proteins known for its infection mechanisms of Creutzfeldt-Jakob disease (CJD) and other diseases. Solved structures and proven biological roles of fungal prions add tremendous potential to conducting computational simulations. Our research focuses on the binding dynamics of HET-s(218-289), one of the heterokaryon fungal prion originated from Podospora anserina, by calculating the binding free energy using umbrella sampling at 300 K. The binding free energy calculated was $-54.5kcal\;mol^{-1}$, relatively similar to the binding energy of other amyloid fibrils. The simulation result suggests the thermodynamic properties of ${\beta}$-solenoid of HET-s prion and its similarity in dissociation pathways compared to amyloids.

• 대한수의사회지
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• 제32권4호
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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.

• BMB Reports
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• 제56권12호
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• pp.645-650
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• 2023

Numerous studies have investigated the cellular prion protein (PrP^C) since its discovery. These investigations have explained that its structure is predominantly composed of alpha helices and short beta sheet segments, and when its abnormal scrapie isoform (PrPSc) is infected, PrPSc transforms the PrP^C, leading to prion diseases, including Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy in cattle. Given its ubiquitous distribution across a variety of cellular types, the PrP^C manifests a diverse range of biological functions, including cell-cell adhesion, neuroprotection, signalings, and oxidative stress response. PrP^C is also expressed in immune tissues, and its functions in these tissues include the activation of immune cells and the formation of secondary lymphoid tissues, such as the spleen and lymph nodes. Moreover, high expression of PrP^C in immune cells plays a crucial role in the pathogenesis of prion diseases. In addition, it affects inflammation and the development and progression of cancer via various mechanisms. In this review, we discuss the studies on the role of PrP^C from various immunological perspectives.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제29권3호
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• pp.262-267
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• 2007

Implantation of allografts has increased widely with not only the availability of many allogenic bone but also allogenic soft tissues. The aim of tissue banking is to provide surgeons with safe tissues compatible with their intended clinical application. The incidence of tissue transplant-transmitted infection is unknown and can only be inferred from prospective studies. The possibility of donor-to-recipient disease transmission through soft tissue transplantation can be considered by reviewing the risk associated with other transplanted hard tissues. Viral, bacterial, and fungal infections have been transmitted via transplantation of soft tissue allografts such as skin, cornea, dura, pericardium. fascia lata, and heart valves. Corneas have transmitted rabies, Creutzfeldt-Jakob disease (CJD), hepatitis B (HBV), cytomegalovirus (CMV), herpes simplex virus (HSV), bacteria, and fungi. Heart valves have been implicated in transmitting tuberculosis, hepatitis B. HIV-1 and CMV. CJD has been transmitted by dura and pericardium transplants. Skin has transmitted CMV, bacteria, and fungi. Cadaveric skin, pericardium, dura, and fascia lata have been used in dental patients with intra-oral soft tissue injuries and GBR. This study is review of the considering transmission of infectious disease in allogenic soft tissues and guidelines of reducing the risk. Prior to use, many tissues are exposed to antibiotics, disinfectants, and sterilants, which further reduce or remove the risk of transmitted disease. Because some soft tissue grafts cannot be subjected to sterilization steps, the risk of infectious disease transmission remains and thorough donor screening and testing is especially important.