Results 61 to 70 of about 3,400,014 (272)

Aspects of prion protein dynamics in cell culture models. [PDF]

, 2005
The cell biology of Prion formation and transfer is not well understood. In order to further elucidate the dynamics of PrPc and PrPsc in a cellular context, fusions between Green Fluorescent Protein (GFP) and PrP were constructed and infected/uninfected ...
Landy, Timothy Adam, Landy, T.A.
core  

Raman optical activity demonstrates poly(L-proline) II helix in the N-terminal region of the ovine prion protein: Implications for function and misfunction [PDF]

, 2004
The aqueous solution structure of the full-length recombinant ovine prion protein PrP25-233, together with that of the N-terminal truncated version PrP94-233, have been studied using vibrational Raman optical activity (ROA) and ultraviolet circular ...
Rhie, A G O, Nielsen, Kurt, Blanch, EW, Rhie, AGO, Gill, AC, Hope, James, Hecht, L, Gill, Andrew C., Gill, A C, Blanch, E W, Barron, Laurence D., Barron, L D, Nielsen, K, Hope, J, Hecht, Lutz, Barron, LD, Blanch, Ewan W., Rhie, Alexandre G. O., Rhie, Alexandre G O   +18 more
core   +1 more source

MARK4 enhances stress granule formation under oxidative stress and increases tau accumulation

FEBS Open Bio, EarlyView.
MARK4 (red dots) localizes to stress granules (orange dots) and promotes their formation under oxidative stress by modulating TIA1 (blue dots). MARK4 and TIA1 synergistically increase tau (purple) accumulation, and the reduction of the TIA1 ortholog suppresses neurodegeneration in a fly model.
Sho Nakajima, Kotone Watanabe, Grigorii Sultanakhmetov, Aoi Fukuchi, Keiya Ito, Sawako Shimizu, Taro Saito, Akiko Asada, Kanae Ando   +8 more
wiley   +1 more source

Loss of Octarepeats in Two Processed Prion Pseudogenes in the Red Squirrel, Sciurus vulgaris [PDF]

, 2010
The N-terminal region of the mammalian prion protein (PrP) contains an 'octapeptide' repeat which is involved in copper binding. This eight- or nine-residue peptide is repeated four to seven times, depending on the species, and polymorphisms in repeat ...
Rheede, T., Marlinda Hupkes, Timothy T. Kortum, Wilfried W. de Jong, Ole Madsen, de Jong, W.W., Kohlen, W., Madsen, O., Jong, W.W., de, Kortum, T.T., Wouter Kohlen, Teun van Rheede, Hupkes, H.   +12 more
core   +1 more source

Prion protein interconversions† [PDF]

Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences, 2001
The transmissible spongiform encephalopathies (TSEs), or prion diseases, remain mysterious neurodegenerative diseases that involve perturbations in prion protein (PrP) structure. This article summarizes our use of in vitro models to describe how PrP is converted to the disease–associated ...
openaire   +2 more sources

Prion Protein Biology [PDF]

Cell, 1998
We thank G. Carlson, N. Nathanson, and J. Safar for carefully reviewing sections of this manuscript. This research was supported by grants from the National Institute of Aging and the National Institute of Neurologic Diseases and Stroke of the National Institutes of Health, International Human Frontiers of Science Program, and American Health ...
Prusiner, Stanley B, Scott, Michael R, DeArmond, Stephen J, Cohen, Fred E   +3 more
openaire   +2 more sources

A high-content neuron imaging assay demonstrates inhibition of prion disease-associated neurotoxicity by an anti-prion protein antibody

Scientific Reports, 2022
There is an urgent need to develop disease-modifying therapies to treat neurodegenerative diseases which pose increasing challenges to global healthcare systems.
Madeleine Reilly, Iryna Benilova, Azadeh Khalili-Shirazi, Christian Schmidt, Parvin Ahmed, Daniel Yip, Parmjit S. Jat, John Collinge   +7 more
doaj   +1 more source

SPG4 and Dementia: Expanding the Clinical Spectrum

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Hereditary spastic paraplegia (HSP) is a group of disorders characterized by progressive spasticity and lower limb weakness, with mutations in SPG4/SPAST being the most common cause. Detailed studies and clinical and molecular comparisons across different populations are missing.
Emanuele Panza, Arun Meyyazhagan, Eliseo Picchi, Gustavo Ribas, Ingrid Faber, Ryosuke Miyamoto, Preethi Basavaraju, Paolo Eusebi, Haripriya Kuchi Bhotla, Mario Stasi, Fabrizio Gaudiello, Francesco Patti, Filippo Maria Santorelli, Marcondes Cavalcante França Jr, José Luiz Pedroso, Orlando Graziani Povoas Barsottini, Hélio Afonso Ghizoni Teive, Peter Henry St George‐Hyslop, Toshitaka Kawarai, Antonio Orlacchio   +19 more
wiley   +1 more source

Synthesis and structural characterization of a mimetic membrane-anchored prion protein [PDF]

, 2006
During pathogenesis of transmissible spongiform encephalopathies (TSEs) an abnormal form (PrPSc) of the host encoded prion protein (PrPC) accumulates in insoluble fibrils and plaques. The two forms of PrP appear to have identical covalent structures, but
Hicks, M R, David H. Crout, Crout, D H, Teresa J. T. Pinheiro, Ian D. Sylvester, Andrew C. Gill, Imanpreet K. Bath, Atvinder K. Rullay, Gill, A C, Bath, I K, Pinheiro, T J T, Matthew R. Hicks, Sylvester, I D, Rullay, A K   +13 more
core   +1 more source

Comprehensive Characterization of 98 Chinese Cases of Genetic Creutzfeldt‐Jakob Disease With T188K Mutation

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To characterize the demographic, clinical, and laboratory features of the Chinese patients of genetic Creutzfeldt‐Jakob disease with T188K variant (T188K‐gCJD), the most common subtype of genetic prion diseases (gPrDs) in China. Methods In this nationwide retrospective study, data from 98 genetically confirmed T188K‐gCJD patients ...
Chun‐Jie Li, Dong‐Lin Liang, Li‐Ping Gao, Wei‐Wei Zhang, Wei Zhou, A. Ruhan, Bing Xu, Run‐Dong Cao, Kang Xiao, Cao Chen, Qi Shi, Xiao‐Ping Dong   +11 more
wiley   +1 more source