Research Topics
| Wen Quan ZouSummaryAffiliation: Case Western Reserve University Country: USA Publications
Research Grants
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Detail Information
Publications
Insoluble cellular prion protein and its association with prion and Alzheimer diseasesWen Quan Zou
Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA
Prion 5:172-8. 2011....
Sporadic fatal insomnia in a young woman: a diagnostic challenge: case reportKaren M Moody
Texas Department of State Health Services, Austin, TX 78756 3199, USA
BMC Neurol 11:136. 2011..Sporadic fatal insomnia (sFI) and fatal familial insomnia (FFI) are rare human prion diseases...
Amyloid-beta42 interacts mainly with insoluble prion protein in the Alzheimer brainWen Quan Zou
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
J Biol Chem 286:15095-105. 2011..Although this work indicated the interaction of Aβ42 with huPrP in the AD brain, the pathophysiological relevance of the iPrP/Aβ42 interaction remains to be established...
PrP conformational transitions alter species preference of a PrP-specific antibodyWen Quan Zou
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio 4410, USA
J Biol Chem 285:13874-84. 2010....
Variably protease-sensitive prionopathy: a new sporadic disease of the prion proteinWen Quan Zou
Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA
Ann Neurol 68:162-72. 2010..The 2 new PSPr forms affect individuals who are either homozygous for methionine (129MM) or heterozygous for methionine/valine (129MV)...
Prion: the chameleon proteinW Q Zou
Department of Pathology and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA
Cell Mol Life Sci 64:3266-70. 2007..Recent progress in modeling prion diseases and characterization of the various prion protein forms reveal that such a wide spectrum of the diseases is associated with the chameleon-like conformational features of prions...
Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristicsIgnazio Cali
Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA
Brain 132:2643-58. 2009..All of these findings indicate that sCJDMM1-2 should be considered as a separate entity at this time...
Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brainsJue Yuan
Department of Pathology and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio 44106, USA
J Biol Chem 281:34848-58. 2006..This is the first study that provides experimental evidence supporting the hypothesis that there might be silent prions lying dormant in normal human brains...
Creutzfeldt-Jakob disease (CJD) with a mutation at codon 148 of prion protein gene: relationship with sporadic CJDManuela Pastore
Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
Am J Pathol 167:1729-38. 2005....
Molecular biology and pathology of prion strains in sporadic human prion diseasesPierluigi Gambetti
Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH, 44106, USA
Acta Neuropathol 121:79-90. 2011..However, the intrinsic characteristics that distinguish at least four of these strains remain to be identified...
Alkylating antitumor drug mechlorethamine conceals a structured PrP domain and inhibits in vitro prion amplificationXiaochen Zhou
Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA
J Toxicol Environ Health A 74:1493-503. 2011..Our findings suggest that MCT may serve as a potential therapeutic agent for prion diseases...
Protease-sensitive prions with 144-bp insertion mutationsXiangzhu Xiao
Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
Aging (Albany NY) 5:155-73. 2013....
A novel human disease with abnormal prion protein sensitive to proteasePierluigi Gambetti
Institute of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
Ann Neurol 63:697-708. 2008....
Characterization of spontaneously generated prion-like conformers in cultured cellsRoger S Zou
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
Aging (Albany NY) 3:968-84. 2011..Our results indicate that iPrPC may be formed more readily in an intracellular compartment and that a significant increase in PrPT183A aggregation may be attributable to the inhibition of autophagy...
Evaluation of the human transmission risk of an atypical bovine spongiform encephalopathy prion strainQingzhong Kong
Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
J Virol 82:3697-701. 2008..Minimal brain spongiosis and long incubation times are observed for the BASE strain-infected Tg mice. These results suggest that in humans, the BASE strain is a more virulent BSE strain and likely lymphotropic...
Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob diseaseWen Quan Zou
Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
J Biol Chem 278:40429-36. 2003..The finding of PrP-CTF12/13 in CJD brains widens the heterogeneity of the PK-resistant PrP fragments associated with prion diseases and may provide useful insights toward the understanding of the PrPSc structure and its formation...
Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United StatesSilvio Notari
Institute of Pathology, Case Western Reserve University, Cleveland, Ohio, United States of America
PLoS ONE 5:e8765. 2010..Three cases have been identified in the United States; however, these subjects were likely exposed to prion infection elsewhere. Here we report on the first of these subjects...
Cellular prion protein is essential for oligomeric amyloid-β-induced neuronal cell deathWataru Kudo
Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
Hum Mol Genet 21:1138-44. 2012..These findings are the first to demonstrate that PrP(C) is required for Aβ oligomer-induced neuronal cell death, the pathology essential to cognitive loss...
Variably protease-sensitive prionopathy: a novel disease of the prion proteinPierluigi Gambetti
Department of Pathology, National Prion Disease Pathology Surveillance Center, School of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA
J Mol Neurosci 45:422-4. 2011..These findings suggest that VPSPr is basically different from classical prion diseases such as sCJD being perhaps more akin to other neurodegenerative dementias...
Glycoform-selective prion formation in sporadic and familial forms of prion diseaseXiangzhu Xiao
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio, United States of America
PLoS ONE 8:e58786. 2013....
Failure to detect the presence of prions in the uterine and gestational tissues from a Gravida with Creutzfeldt-Jakob diseaseXiangzhu Xiao
Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
Am J Pathol 174:1602-8. 2009..Nevertheless, examination by a highly sensitive bioassay is ongoing to ascertain possible prion infectivity from CJD in the amniotic fluid...
Creutzfeldt-Jakob disease with unusually extensive neuropathology in a child treated with native human growth hormoneJacqueline Mikol
Denis Diderot University
Clin Neuropathol 31:127-34. 2012..A transitional form of the disease between common iatrogenic and panencephalopathic CJD is suggested...
Accessibility of a critical prion protein region involved in strain recognition and its implications for the early detection of prionsJ Yuan
Department of Pathology and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA
Cell Mol Life Sci 65:631-43. 2008..Our study suggests that an increase in the level of PrP*20 characterizes the early stages of prion diseases...
All or none fibrillogenesis of a prion peptideW Q Zou
Centre for Research in Neurodegenerative Diseases, University of Toronto, Ontario, Canada
Eur J Biochem 268:4885-91. 2001..This variation in whether oligomeric intermediates are stably populated during fibril formation indicates that amyloid proteins assemble into a common fibrillar structure; however, they do so through different pathways...
Research Grants
- Pathogenicity of prion protein aggregates from uninfected cells and brainsWen Quan Zou; Fiscal Year: 2009..The proposed study will improve our understanding of PrPSc formation in the spontaneous prion diseases, and facilitate the development of early diagnostic and therapeutic strategies. ..
- Pathogenicity of prion protein aggregates from uninfected cells and brainsWen Quan Zou; Fiscal Year: 2010..The proposed study will improve our understanding of PrPSc formation in the spontaneous prion diseases, and facilitate the development of early diagnostic and therapeutic strategies. ..
