Neurofilament light chain and tau concentrations are markedly increased in the serum of patients with sporadic Creutzfeldt-Jakob disease, and tau correlates with rate of disease progression [PDF]
OBJECTIVES: A blood-based biomarker of neuronal damage in sporadic Creutzfeldt-Jakob disease (sCJD) will be extremely valuable for both clinical practice and research aiming to develop effective therapies.
Collinge, J +6 more
core +1 more source
Involvement of N- and C-terminal region of recombinant cervid prion protein in its reactivity to CWD and atypical BSE prions in real-time quaking-induced conversion reaction in the presence of high concentrations of tissue homogenates. [PDF]
Suzuki A +6 more
europepmc +2 more sources
Early and Pre-Clinical Detection of Prion Seeding Activity in Cerebrospinal Fluid of Goats using Real-Time Quaking-Induced Conversion Assay. [PDF]
Favole A +15 more
europepmc +3 more sources
Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems [PDF]
Dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) can be divided into two subgroups that exhibit distinct clinical and neuropathological features, with the majority represented by a non-plaque-type of dCJD (np-dCJD) and the minority by a ...
Atsushi Kobayashi +3 more
core +1 more source
Prion Seeding Activities of Mouse Scrapie Strains with Divergent PrPSc Protease Sensitivities and Amyloid Plaque Content Using RT-QuIC and eQuIC [PDF]
Different transmissible spongiform encephalopathy (TSE)-associated forms of prion protein (e.g. PrPSc) can vary markedly in ultrastructure and biochemical characteristics, but each is propagated in the host. PrPSc propagation involves conversion from its
AH Peden +70 more
core +3 more sources
Prion disease: experimental models and reality [PDF]
The understanding of the pathogenesis and mechanisms of diseases requires a multidisciplinary approach, involving clinical observation, correlation to pathological processes, and modelling of disease mechanisms.
Brandner, S, Jaunmuktane, Z
core +1 more source
Imaging and CSF analyses effectively distinguish CJD from its mimics [PDF]
OBJECTIVE: To review clinical and investigation findings in patients referred to a specialist prion clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alternative final diagnosis. METHODS: Review the
Collinge, J +4 more
core +1 more source
An autopsy-verified case of steroid-responsive encephalopathy with convulsion and a false-positive result from the real-time quaking-induced conversion assay. [PDF]
Hayashi Y +9 more
europepmc +2 more sources
CJD mimics and chameleons [PDF]
Rapidly progressive dementia mimicking Creutzfeldt-Jakob disease (CJD) is a relatively rare presentation but a rewarding one to become familiar with, as the potential diagnoses range from the universally fatal to the completely reversible.
Mead, S, Rudge, P
core +1 more source
Early preclinical detection of prions in the skin of prion-infected animals
There are currently no validated methods for the diagnosis of prion disease at the preclinical stage. Here the authors show that serial protein misfolding cyclic amplification and real-time quaking-induced conversion can be used to detect prions in the ...
Zerui Wang +24 more
doaj +1 more source

