Results 41 to 50 of about 45,606 (277)

Human prion diseases and the prion protein – what is the current state of knowledge?

open access: yesTranslational Neuroscience, 2023
Prion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge.
Nafe Reinhold   +2 more
doaj   +1 more source

Posterior Cortical Atrophy in the Asia‐Pacific: A Report From the PCA Asian Workgroup

open access: yesAnnals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Posterior Cortical Atrophy (PCA) is a distinct dementia syndrome primarily affecting spatial abilities and visual processing. It is associated with degeneration in the posterior part of the brain. PCA is subclassified into PCA‐pure and PCA‐plus syndromes based on consensus criteria.
Yuttachai Likitjaroen   +11 more
wiley   +1 more source

Treatment of Prion Disease with Heterologous Prion Proteins. [PDF]

open access: yesPLoS ONE, 2015
Prion diseases such as Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy in cattle, and scrapie in sheep are fatal neurodegenerative diseases for which there is no effective treatment.
Pamela J Skinner   +9 more
doaj   +1 more source

SPG4 and Dementia: Expanding the Clinical Spectrum

open access: yesAnnals 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   +19 more
wiley   +1 more source

Anti-prion activity of Brilliant Blue G. [PDF]

open access: yesPLoS ONE, 2012
BACKGROUND: Prion diseases are fatal neurodegenerative disorders with no effective therapy currently available. Accumulating evidence has implicated over-activation of P2X7 ionotropic purinergic receptor (P2X7R) in the progression of neuronal loss in ...
Yoshifumi Iwamaru   +9 more
doaj   +1 more source

SERPINA3/SerpinA3n: a novel promising therapeutic target in prion diseases [PDF]

open access: yes, 2021
Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), belong to a class of fatal neurodegenerative disorders characterized by vacuolation and neuronal loss in the brain paralleled by cognitive and motor impairments.
Colini Baldeschi, Arianna
core  

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

open access: yesAnnals 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   +11 more
wiley   +1 more source

Neuropathology of Animal Prion Diseases

open access: yesBiomolecules, 2021
Transmissible Spongiform Encephalopathies (TSEs) or prion diseases are a fatal group of infectious, inherited and spontaneous neurodegenerative diseases affecting human and animals.
Leonor Orge   +21 more
doaj   +1 more source

Characterization of the prion protein in relation to normal cellular function and in disease [PDF]

open access: yes, 2012
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of rare and fatal neurodegenerative disorders that can affect both human and animals.
Wik, Lotta
core  

A study of the incubation period, or age at onset, of the transmissible spongiform encephalopathies/prion diseases. [PDF]

open access: yes
In order to model epidemics of infectious diseases, particularly to estimate probable numbers of cases with onset at any particular time, it is necessaiy to incorporate a term for the incubation period frequency distribution. Sartwell's hypothesis states
Wooldridge, Marion Joan Anstee
core   +1 more source

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