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Analysis of 12 Chinese Patients with Proline-to-Leucine Mutation at Codon 102-Associated Gerstmann-Sträussler-Scheinker Disease

Wang J, Xiao K, Zhou W, Shi Q, Dong XP

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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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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Amplification of distinct α-synuclein fibril conformers through protein misfolding cyclic amplification

Jung BC, Lim YJ, Bae EJ, Lee JS, Choi MS, Lee MK, Lee HJ, Kim YS, Lee SJ

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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Classical natural ovine scrapie prions detected in practical volumes of blood by lamb and transgenic mouse bioassays

Dassanayake RP, Truscott T, Zhuang D, Schneider , Madsen-Bouterse SA, Young AJ, Stanton JB, Davis WC, O'Rourke K

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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Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key?

Lee S, Kim HJ

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

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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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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Characterizing affinity epitopes between prion protein and beta-amyloid using an epitope mapping immunoassay

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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Pathological characterization of TgElk mice injected with brain homogenate from elk with chronic wasting disease

Jeon YC, Choi JK, Choi EK, Carp RI, Kim YS

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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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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A Case of Familial Creutzfeldt-Jacob Disease (V180I) Initially Presenting with Depression

Joo J, Yang Y, Kang JH, Lee SH, Ha SW, Han JH, Cho EK, Kim DE

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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Sequence variations of the bovine prion protein gene (PRNP) in native Korean Hanwoo cattle

Choi S, Woo HJ, Lee J

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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Dura Mater Graft-Associated Creutzfeldt-Jakob Disease: The First Case in Korea

Kim HL, Do JY, Cho HJ, Jeon YC, Park SJ, Ma HI, Song JH, Lee Y, Choi H, Choi KC, Kim YS, Zerr I, Kallenberg K, Kim YJ

Since 1987, dura mater graft-associated iatrogenic Creutzfeldt-Jakob disease (dCJD) has been reported in many countries. We report the first case of dCJD in Korea. A 54-yr-old woman, who underwent resection...
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Familial Creutzfeldt-Jakob Disease with V180I Mutation

Yang TI, Jung DS, Ahn BY, Jeong BH, Cho HJ, Kim YS, Na DL, Geschwind , Kim EJ

Creutzfeldt-Jakob disease (CJD) is an uncommon neurodegenerative disorder with an incidence of 1 per 1000,000 per year typically characterized by rapidly progressive dementia, ataxia, myoclonus and behavioral changes. Genetic prion...
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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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A Case of Heidenhain Variant of Sporadic Creutzfeldt-Jakob Disease

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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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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Identification of single-nucleotide polymorphisms of the prion protein gene in sika deer (Cervus nippon laiouanus)

Jeong HJ, Lee JB, Park SY, Song CS, Kim BS, Rho JR, Yoo MH, Jeong YH, Kim YS, Choi IS

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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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

Kahraman M, Ozygit MO, Akkoc A, Ediz B, Misirlioglu D, Sonmez G, Alasonyalilar A, Yilmaz R

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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Biological characteristics of Chinese hamster ovary cells transfected with bovine Prnp

Kang SG, Lee DY, Kang ML, Yoo HS

A normal prion protein (PrPc) is converted to a proteaseresistant isoform by an apparent self-propagating activity in transmissible spongiform encephalopathy, a neurodegenerative disease. The cDNA encoding open reading frame (ORF)...
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