Results 91 to 100 of about 51,393 (297)

Human Prion Disease and Human Prion Protein Disease [PDF]

, 1996
Creutzfeldt-Jakob disease (CJD), kuru, and Gerstmann-Straussler syndrome (GSS) show clinical and pathological characteristics similar to those of scrapie, a transmissible neurodegenerative disease of sheep and goats. These diseases are caused by slow infectious agents designated as prions (PRUSINER 1982). The major component of prions is prion protein (
T, Kitamoto, J, Tateishi
openaire   +2 more sources

Prion degradation pathways: Potential for therapeutic intervention

, 2015
Prion diseases are fatal neurodegenerative disorders. Pathology is closely linked to the misfolding of native cellular PrP(C) into the disease-associated form PrP(Sc) that accumulates in the brain as disease progresses. Although treatments have yet to be
McKinnon, C, Tabrizi, SJ, Goold, R
core  

SERPINA3/SerpinA3n role in prion diseases [PDF]

, 2021
Prion diseases are a family of rare and fatal neurodegenerative disorders, characterized by the accumulation of abnormally folded prion protein. Previous data suggested that SERPINA3/SerpinA3n might be involved in the pathogenesis and the progression of ...
Zattoni, Marco
core  

Co‐ and Multi‐Pathologies in Parkinson's Disease: An International Parkinson and Movement Disorder Society Scientific Issues Committee Review

Movement Disorders, EarlyView.
Abstract Parkinson's disease (PD) has been historically defined as a disease of striatal dopamine deficiency secondary to degeneration of dopaminergic neurons in the substantia nigra pars compacta, related to the presence of Lewy bodies and Lewy neurites.
Michele Matarazzo, Per Borghammer, Inas Elsayed, Jennifer G. Goldman, Yue Huang, Katja Lohmann, Per Svenningsson, Lorraine V. Kalia, Daniela Berg, Jeffrey H. Kordower, the MDS Scientific Issues Committee   +10 more
wiley   +1 more source

PrPCWD lymphoid cell targets in early and advanced chronic wasting disease of mule deer

, 2002
Up to 15% of free-ranging mule deer in northeastern Colorado and southeastern Wyoming, USA, are afflicted with a prion disease, or transmissible spongiform encephalopathy (TSE), known as chronic wasting disease (CWD).
Keulen, L.J.M., van, van Keulen, L.J.M., Miller, M.W., Oesch, B., Hoover, E.A., Langeveld, J.P.M., Sigurdson, C.J., Barillas-Mury, C.   +7 more
core   +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

Spatiotemporal Progression Patterns of Striatal Dopamine Depletion and Cerebral Hypoperfusion in Parkinson's Disease

Movement Disorders, EarlyView.
Abstract Background The identification of Parkinson's disease (PD) subtypes is crucial for predicting the disease course and designing personalized therapeutic strategies. Objectives The aim of the study was to characterize the heterogeneity of the spatiotemporal evolutionary patterns of striatal dopamine depletion and cerebral hypoperfusion in PD ...
Yeeun Sun, Youngseok Choi, Chan Wook Park, Han Kyu Na, Hye Sun Lee, Yun Joong Kim, Young H. Sohn, Phil Hyu Lee, Jeyeon Lee, Seok Jong Chung   +9 more
wiley   +1 more source

A rapid dual staining procedure for the quantitative discrimination of prion amyloid from tissues reveals how interactions between amyloid and lipids in tissue homogenates may hinder the detection of prions

, 2009
Transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases with no cure to this day, and are often associated with the accumulation of amyloid plaques in the brain and other tissues in affected individuals. The emergence of new
Keevil, C.William, Collin, R., Hervé, R., Secker, Thomas, Pinchin, H.E.   +4 more
core   +1 more source

Differentiation of Plant and Animal‐Derived Cholesterol Using irm‐13C NMR and IRMS

Magnetic Resonance in Chemistry, EarlyView.
Determining and certifying the origin of ingredients, starting materials, and excipients used in manufactured goods like cosmetics and medicines can be difficult. In this report, we describe a robust approach for identifying the origin of cholesterol, a component of myriad consumer products using irm‐13C NMR and IRMS to differentiate plant versus ...
Anika M. Singh, Subir Chakraborty, Remington X. Poulin, R. Thomas Williamson   +3 more
wiley   +1 more source

Characterization of variably protease-sensitive prionopathy by capillary electrophoresis

Scientific Reports
Variably Protease Sensitive Prionopathy (VPSPr) is a rare human prion disease that, like Creutzfeldt-Jakob disease (CJD), results in the deposition of abnormally folded prion protein aggregates in the brain and is ultimately fatal.
Jennifer Myskiw, Ben A. Bailey-Elkin, Kristen Avery, Marcelo A. Barria, Diane L. Ritchie, Mark L. Cohen, Brian S. Appleby, Stephanie A Booth   +7 more
doaj   +1 more source