- Prions as Proteinaceous Infectious Particles
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Yang SM, Lee WK
- KMID: 1892738
- Korean J Clin Microbiol.
- 2000 Sep;3(2):89-93.
No abstract available. -
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- Prion; the unconventional slow infectious agent
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Kim YS
- KMID: 645022
- J Korean Med Sci.
- 1997 Feb;12(1):1-8.
- doi: 10.3346/jkms.1997.12.1.1
No abstract available. -
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- Evaluation of Infective Property of Recombinant Prion Protein Amyloids in Cultured Cells Overexpressing Cellular Prion Protein
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Kim DH, Lee HM, Ryou C
- KMID: 2069947
- J Korean Med Sci.
- 2014 Dec;29(12):1604-1609.
- doi: 10.3346/jkms.2014.29.12.1604
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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- Gerstmann-Sträussler-Scheinker Disease (Pro102Leu) Presenting as Rapidly Progressive Dementia
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Jung SH, Chae SH, Hwangbo J, Kim HS, Lee YJ, Kim YS, Jung NY
- KMID: 2464144
- J Korean Neurol Assoc.
- 2019 Nov;37(4):384-387.
- doi: 10.17340/jkna.2019.4.9
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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- Human Prion Diseases
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Kim SY, Cheong HK, An SS
- KMID: 2185973
- J Korean Med Assoc.
- 2008 Dec;51(12):1125-1138.
- doi: 10.5124/jkma.2008.51.12.1125
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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- Classical natural ovine scrapie prions detected in practical volumes of blood by lamb and transgenic mouse bioassays
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Dassanayake RP, Truscott T, Zhuang D, Schneider , Madsen-Bouterse SA, Young AJ, Stanton JB, Davis WC, O'Rourke K
- KMID: 2164527
- J Vet Sci.
- 2015 Jun;16(2):179-186.
- doi: 10.4142/jvs.2015.16.2.179
Scrapie is diagnosed antemortem in sheep by detecting misfolded isoforms of prion protein (PrP(Sc)) in lymphoid follicles of the rectal mucosa and nictitating membranes. Assay sensitivity is limited if (a)... -
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- Amplification of distinct α-synuclein fibril conformers through protein misfolding cyclic amplification
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Jung BC, Lim YJ, Bae EJ, Lee JS, Choi MS, Lee MK, Lee HJ, Kim YS, Lee SJ
- KMID: 2382705
- Exp Mol Med.
- 2017 Apr;49(4):e314.
- doi: 10.1038/emm.2017.1
Amyloid fibril formation has been implicated in the pathogenesis of neurodegenerative diseases. Fibrillation generates numerous conformers. Presumably, the conformers may possess specific biological properties, thus providing a biochemical framework for... -
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- A Case of Familial Creutzfeldt-Jacob Disease (V180I) Initially Presenting with Depression
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Joo J, Yang Y, Kang JH, Lee SH, Ha SW, Han JH, Cho EK, Kim DE
- KMID: 2172239
- Dement Neurocogn Disord.
- 2012 Jun;11(2):74-77.
- doi: 10.12779/dnd.2012.11.2.74
Creutzfeldt-Jakob disease (CJD) is a degenerative neurological disorder that is incurable and invariably fatal. It is characterized by rapidly progressive dementia presenting with memory loss, personality changes and hallucinations. The... -
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- A Case of Heidenhain Variant of Sporadic Creutzfeldt-Jakob Disease
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Kim DH, Kim SY, Yang YS, Jeong SH, Kim JS, Hwang JM
- KMID: 2337675
- J Korean Ophthalmol Soc.
- 2008 Sep;49(9):1548-1552.
PURPOSE: To report a case of the Heidenhain variant of sporadic Creutzfeldt-Jakob disease (CJD), predominantly characterized by visual impairment at onset. CASE SUMMARY: History-taking, ophthalmologic examination, neurologic examination, cerebrospinal fluid examination... -
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- Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key?
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Lee S, Kim HJ
- KMID: 2052846
- Exp Neurobiol.
- 2015 Mar;24(1):1-7.
- doi: 10.5607/en.2015.24.1.1
ALS is a fatal adult-onset motor neuron disease. Motor neurons in the cortex, brain stem and spinal cord gradually degenerate in ALS patients, and most ALS patients die within 3~5... -
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- Genetic Studies in Human Prion Diseases
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Jeong BH, Kim YS
- KMID: 1786101
- J Korean Med Sci.
- 2014 May;29(5):623-632.
- doi: 10.3346/jkms.2014.29.5.623
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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- Pathological characterization of TgElk mice injected with brain homogenate from elk with chronic wasting disease
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Jeon YC, Choi JK, Choi EK, Carp RI, Kim YS
- KMID: 1482809
- J Vet Sci.
- 2013 Mar;14(1):21-26.
- doi: 10.4142/jvs.2013.14.1.21
Chronic wasting disease (CWD) is classified as a transmissible spongiform encephalopathy or prion disease that affects cervids. CWD has been reported in 15 US states, two Canadian provinces, and in... -
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- Characterizing affinity epitopes between prion protein and beta-amyloid using an epitope mapping immunoassay
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Kang M, Kim SY, An SS, Ju YR
- KMID: 2154253
- Exp Mol Med.
- 2013 Aug;45(8):e34.
Cellular prion protein, a membrane protein, is expressed in all mammals. Prion protein is also found in human blood as an anchorless protein, and this protein form is one of... -
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- Analysis of 12 Chinese Patients with Proline-to-Leucine Mutation at Codon 102-Associated Gerstmann-Sträussler-Scheinker Disease
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Wang J, Xiao K, Zhou W, Shi Q, Dong XP
- KMID: 2467732
- J Clin Neurol.
- 2019 Apr;15(2):184-190.
- doi: 10.3988/jcn.2019.15.2.184
BACKGROUND AND PURPOSE: Gerstmann-Sträussler-Scheinker disease (GSS) with a proline-to-leucine mutation at codon 102 (P102L) in the PRNP gene is the most frequently reported GSS subtype worldwide. This study aimed to... -
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- Comparative bioinformatics analysis of prion proteins isolated from reptile, rodent, ruminant, and human species
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Ahn I, Son HS
- KMID: 1091071
- Exp Mol Med.
- 2007 Dec;39(6):769-777.
Prion proteins (PrPs) are infectious pathogens that cause a group of invariably fatal, neurodegenerative diseases, including Creutzfeldt-Jakob disease, by means of an entirely novel mechanism. They are produced by various... -
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- Discriminant analysis of prion sequences for prediction of susceptibility
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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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- Targeted surveillance to assess the presence of BSE in the age risk population of cattle slaughtered in Bursa, Turkey: preliminary results of an immunohistochemical detection study for the 2004-2005 period
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Kahraman M, Ozygit MO, Akkoc A, Ediz B, Misirlioglu D, Sonmez G, Alasonyalilar A, Yilmaz R
- KMID: 1089673
- J Vet Sci.
- 2007 Jun;8(2):193-195.
- doi: 10.4142/jvs.2007.8.2.193
Bovine spongiform encephalopathy (BSE), a member of the transmissible spongiform encepahlopathies, has been a notifiable disease in Turkey since 1997. In 2002, the BSE status of Turkey was assessed by... -
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- Molecular analysis of prion protein gene (PRNP) in Korean patients with Creutzfeldt-Jakob disease
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Jeong BH, Ju WK, Huh K, Lee EA, Choi IS, Im JH, Choi EK, Kim YS
- KMID: 1745544
- J Korean Med Sci.
- 1998 Jun;13(3):234-240.
- doi: 10.3346/jkms.1998.13.3.234
Creutzfeldt-Jakob disease (CJD), a relatively uncommon human dementia, is caused by an unconventional slow infectious agent. Several cases of CJD, clinically or histopathologically diagnosed, have been reported in Korea.... -
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- Identification of single-nucleotide polymorphisms of the prion protein gene in sika deer (Cervus nippon laiouanus)
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Jeong HJ, Lee JB, Park SY, Song CS, Kim BS, Rho JR, Yoo MH, Jeong YH, Kim YS, Choi IS
- KMID: 1090807
- J Vet Sci.
- 2007 Sep;8(3):299-301.
- doi: 10.4142/jvs.2007.8.3.299
Polymorphisms of the prion protein gene (PRNP) havebeen detected in several cervid species. In order toconfirm the genetic variations, this study examined theDNA sequences of the PRNP obtained from 33... -
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- Sequence variations of the bovine prion protein gene (PRNP) in native Korean Hanwoo cattle
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Choi S, Woo HJ, Lee J
- KMID: 1376186
- J Vet Sci.
- 2012 Jun;13(2):127-137.
- doi: 10.4142/jvs.2012.13.2.127
Bovine spongiform encephalopathy (BSE) is one of the fatal neurodegenerative diseases known as transmissible spongiform encephalopathies (TSEs) caused by infectious prion proteins. Genetic variations correlated with susceptibility or resistance to... -
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