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Prion; the unconventional slow infectious agent

Kim YS

No abstract available.
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Prion Diseases

Kim YS

  • KMID: 1812909
  • J Korean Neurol Assoc.
  • 2001 Jan;19(1):1-9.
No abstract available.
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Human Prion Diseases

Kim SY, Cheong HK, An SS

Transmissible Spongiform Encephalopathy (TSE) or prion diseases are fatal neurodegenerative diseases, which are caused by transmissible abnormal prion proteins, converting the endogenous normal prion in the body to the infectious...
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Genetic Studies in Human Prion Diseases

Jeong BH, Kim YS

Human prion diseases are fatal neurodegenerative disorders that are characterized by spongiform changes, astrogliosis, and the accumulation of an abnormal prion protein (PrP(Sc)). Approximately 10%-15% of human prion diseases are...
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Increases in the Proteins Modified by Malondialdehyde and Hydroxynonenal in the Hippocampus of Prion-Infected Mice

Kim JI, Lee HG

Prion diseases, also termed transmissible spongiform encephalopathies (TSEs), are rare and fatal neurodegenerative conditions that affect both humans and animals. Although there is increased evidence that oxidative stress plays an...
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Discriminant analysis of prion sequences for prediction of susceptibility

Lee JH, Bae SE, Jung S, Ahn I, Son HS

  • KMID: 2154267
  • Exp Mol Med.
  • 2013 Oct;45(10):e48.
Prion diseases, including ovine scrapie, bovine spongiform encephalopathy (BSE), human kuru and Creutzfeldt-Jakob disease (CJD), originate from a conformational change of the normal cellular prion protein (PrPC) into abnormal protease-resistant...
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Gerstmann-Sträussler-Scheinker Disease (Pro102Leu) Presenting as Rapidly Progressive Dementia

Jung SH, Chae SH, Hwangbo J, Kim HS, Lee YJ, Kim YS, Jung NY

Genetic prion diseases account for about 10-15% of all cases of human prion disease and are caused by mutations in the prion protein gene. Gerstmann-Sträussler-Scheinker (GSS) disease is a rare...
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Evaluation of Infective Property of Recombinant Prion Protein Amyloids in Cultured Cells Overexpressing Cellular Prion Protein

Kim DH, Lee HM, Ryou C

Misfolded isoform of prion protein (PrP), termed scrapie PrP (PrP(Sc)), tends to aggregate into various fibril forms. Previously, we reported various conditions that affect aggregation of recombinant PrP into amyloids....
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Rapidly Aggravated Creutzfeldt-Jacob Disease: Autopsy-Proven Case

Park SH, Kang HK, Yu H, Lee SC

Creutzfeldt-Jakob disease (CJD) is one of the transmissible spongiform encephalopathies, which is mediated by what has been known as "prion". It is a rare and fatal progressive neurodegenerative disease that...
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A Case of Creutzfeldt-Jakob Disease Presenting Mainly with Abnormal Eye Movements

Kang YJ, Kim JH, Kim TE, Park SA, Lee TK

  • KMID: 1910485
  • Res Vestib Sci.
  • 2014 Jun;13(2):53-56.
Creutzfeldt-Jakob disease (CJD) is a human prion disease with rapidly progressive neurodegeneration. The major clinical manifestations of CJD include mental deterioration, myoclonus, cerebellar dysfunction, and neuro-ophthalmic symptoms and signs. However,...
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Re-transmissibility of mouse-adapted ME7 scrapie strain to ovine PrP transgenic mice

Babalola J, Kim JM, Lee YJ, Park JH, Choi HS, Choi YG, Choi EK, Kim YS

Scrapie is a mammalian transmissible spongiform encephalopathy or prion disease that predominantly affects sheep and goats. Scrapie has been shown to overcome the species barrier via experimental infection of other...
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Real-Time Quaking-Induced Conversion Analysis for the Diagnosis of Sporadic Creutzfeldt-Jakob Disease in Korea

Park JH, Choi YG, Lee YJ, Park SJ, Choi HS, Choi KC, Choi EK, Kim YS

BACKGROUND AND PURPOSE: The level of 14-3-3 protein in the cerebrospinal fluid (CSF) is increased in Creutzfeldt-Jakob disease (CJD) patients, which has led to it being used as a clinical...
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Ubiquitin C-terminal Hydrolase L1 Regulates Lipid Raft-dependent Endocytosis

Kang SJ, Kim JS, Park SM

Ubiquitin C-terminal hydrolase L1 (UCH-L1) is a deubiquitinating enzyme that is highly expressed in neurons, and gathering evidence indicates that UCH-L1 may play pathogenic roles in many neurodegenerative disorders such...
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The Effect of Compressed Air Jet Cleaning on the Elimination of Postoperative Residual Protein from a ProSeal(TM) Laryngeal Mask Airway

Choi WJ, Kim YH, Kyoung MK

BACKGROUND: The reusable ProSeal(TM) laryngeal mask airways (PLMA's) have the potential to act as a vector for the transmission of prion diseases such as variant Creutzveldt-Jacob disease. This study tested...
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Neurologists' Awareness and Preparedness on Prion Diseases in Korea

Jang JW, Park YH, Lim JS, Park SC, Cheong HK, Kim JE, Kim S

BACKGROUND: Creutzfeldt-Jakob disease (CJD) is very rare human prion disease. But, neurologists take a key role in diagnosis, surveillance and management of the cases because of its complexity and difficulty...
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A Case of Gerstmann-Straussler-Scheinker Disease

Park MJ, Jo HY, Cheon SM, Choi SS, Kim YS, Kim JW

BACKGROUND: Gerstmann-Straussler-Scheinker disease (GSS) is a type of human transmissible spongiform encephalopathy (TSE) that is determined genetically. CASE REPORT: A 46-year-old woman presented with a slowly progressive ataxic gait and cognitive...
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The effect of ultrasonic cleaning on the elimination of postoperative residual protein from the proseal(TM) laryngeal mask airway

Choi WJ, Kim YH, Kim HS, Ryu KH

BACKGROUND: Reusable Proseal(TM) laryngeal mask airways (PLMAs) can act as a vector for the transmission of prion diseases such as variant Creutzfeldt-Jacob disease. This study tested the hypothesis that supplementary...
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Estimation of the size of the iatrogenic Creutzfeldt-Jakob disease outbreak associated with cadaveric dura mater grafts in Korea

Jeon BH, Kim J, Kim G, Park S, Kim S, Cheong HK

OBJECTIVES: This study estimated the overall incidence of iatrogenic Creutzfeldt-Jakob disease (iCJD) based on dura graft cases in Korea using a mathematical model. METHODS: We estimated the number of annual dura...
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Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies

Ciechanover A, Kwon YT

Mammalian cells remove misfolded proteins using various proteolytic systems, including the ubiquitin (Ub)-proteasome system (UPS), chaperone mediated autophagy (CMA) and macroautophagy. The majority of misfolded proteins are degraded by the...
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Genetic variability of the prion protein gene (PRNP) in wild ruminants from Italy and Scotland

Peletto S, Perucchini M, Acin C, Dalgleish , Reid HW, Rasero R, Sacchi P, Stewart P, Caramelli , Ferroglio , Bozzetta E, Meloni D, Orusa R, Robetto S, Gennero S, Goldmann W, Acutis PL

The genetics of the prion protein gene (PRNP) play a crucial role in determining the relative susceptibility to transmissible spongiform encephalopathies (TSEs) in several mammalian species. To determine the PRNP...
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