2013
DOI: 10.1371/journal.pone.0060003
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Relationships between Clinicopathological Features and Cerebrospinal Fluid Biomarkers in Japanese Patients with Genetic Prion Diseases

Abstract: A national system for surveillance of prion diseases (PrDs) was established in Japan in April 1999. Here, we analyzed the relationships among prion protein gene (PRNP) mutations and the clinical features, cerebrospinal fluid (CSF) markers, and pathological characteristics of the major genotypes of genetic PrDs (gPrDs). We retrospectively analyzed age at onset and disease duration; the concentrations and incidences of 14-3-3 protein, tau protein, and abnormal prion protein (PrPSc) in the CSF of 309 gPrD patient… Show more

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Cited by 38 publications
(98 citation statements)
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“…In this cohort, western blotting of brain homogenates from patients with V180I indicated only weak bands of the monoglycosylated and unglycosylated fragments 5. In the future, studies should examine the role of factors that influence lesion topology on the disease's clinical expression and progression.…”
Section: Discussionmentioning
confidence: 79%
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“…In this cohort, western blotting of brain homogenates from patients with V180I indicated only weak bands of the monoglycosylated and unglycosylated fragments 5. In the future, studies should examine the role of factors that influence lesion topology on the disease's clinical expression and progression.…”
Section: Discussionmentioning
confidence: 79%
“…We evaluated 14-3-3 and total τ (t-τ) protein levels in the CSF by western blotting as previously described 5. PrP Sc in the CSF was detected by real-time quaking induced conversion (RT-QUIC), as previously described 12.…”
Section: Methodsmentioning
confidence: 99%
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“…Similarly, several neuropathological features that are associated with the V180I‐129M/PrP Sc type 2 combination, including the florid spongiform change lacking confluent vacuoles, as well as the faint synaptic type pattern of PrP immunostaining, do not fully match those of the sCJDMM2 subtypes .…”
Section: Phenotypic Spectrum and Classification Of Disease Subtypesmentioning
confidence: 98%