Genetic PrP prion diseases

MO Kim, LT Takada, K Wong… - Cold Spring …, 2018 - cshperspectives.cshlp.org
Genetic prion diseases (gPrDs) caused by mutations in the prion protein gene (PRNP) have
been classified as genetic Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker …

Characterization of mutations in PRNP (prion) gene and their possible roles in neurodegenerative diseases

E Bagyinszky, VV Giau, YC Youn, SSA An… - … disease and treatment, 2018 - Taylor & Francis
Abnormal prion proteins are responsible for several fatal neurodegenerative diseases in
humans and in animals, including Creutzfeldt–Jakob disease (CJD), Gerstmann–Sträussler …

Genetic prion disease: experience of a rapidly progressive dementia center in the United States and a review of the literature

LT Takada, MO Kim, RW Cleveland… - American Journal of …, 2017 - Wiley Online Library
Although prion diseases are generally thought to present as rapidly progressive dementias
with survival of only a few months, the phenotypic spectrum for genetic prion diseases …

Genetic Creutzfeldt–Jakob disease and fatal familial insomnia: insights into phenotypic variability and disease pathogenesis

S Capellari, R Strammiello, D Saverioni… - Acta …, 2011 - Springer
Human prion diseases are a group of rare neurodegenerative disorders characterized by
the conversion of the constitutively expressed prion protein, PrP C, into an abnormally …

Genetic Creutzfeldt–Jakob disease

A Ladogana, GG Kovacs - Handbook of Clinical Neurology, 2018 - Elsevier
Abstract Genetic Creutzfeldt–Jakob disease (CJD) is associated with mutations in the
human PrP gene (PRNP) on chromosome 20p12-pter. Pathogenic mutations have been …

The features of genetic prion diseases based on Chinese surveillance program

Q Shi, W Zhou, C Chen, BY Zhang, K **ao, XC Zhang… - PloS one, 2015 - journals.plos.org
Objective To identify the features of Chinese genetic prion diseases. Methods Suspected
Creutzfeldt-Jakob disease (CJD) cases that were reported under CJD surveillance were …

Prion protein: orchestrating neurotrophic activities

VR Martins, FH Beraldo, GN Hajj, MH Lopes… - Current issues in …, 2010 - mdpi.com
PrP C is highly expressed in both the central and peripheral nervous systems from early
stages of development and in adulthood. Its major conformational change and conversion …

The epidemiological, clinical, and laboratory features of sporadic Creutzfeldt-Jakob disease patients in China: surveillance data from 2006 to 2010

C Gao, Q Shi, C Tian, C Chen, J Han, W Zhou… - PloS one, 2011 - journals.plos.org
Background Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive fatal central
nervous system disorder, which consists of three main catalogues: sporadic, familial, and …

Prion disease

LT Takada, MO Kim, S Metcalf, II Gala… - Handbook of Clinical …, 2018 - Elsevier
Genetic prion diseases (gPrDs) are caused by autosomal-dominant mutations in the prion
protein gene (PRNP). Although the first PRNP mutations identified, and most since, are …

[HTML][HTML] Characteristics of different types of prion diseases—China's surveillance

Q Shi, C Chen, K **ao, W Zhou, C Gao, L Gao… - China CDC …, 2022 - ncbi.nlm.nih.gov
This report briefly described the establishment and implementation of national surveillance
for human prion disease (PrD) in China. Reported cases came from Chinese surveillance …