PrP knock-out and PrP transgenic mice in prion research
نویسندگان
چکیده
منابع مشابه
PrP knock-out and PrP transgenic mice in prion research.
Spongiform encephalopathies such as scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle or Creutzfeldt-Jacob disease (CJD) and Gerstmann-Sträussler-Scheinker syndrome (GSS) in humans is caused by a transmissible agent designated prion. The 'protein only' hypothesis proposes that the prion consists partly or entirely of a conformational isoform of the normal host protein PrP(C), d...
متن کاملPrion Protein (PrP) Knock-Out Mice Show Altered Iron Metabolism: A Functional Role for PrP in Iron Uptake and Transport
Despite overwhelming evidence implicating the prion protein (PrP) in prion disease pathogenesis, the normal function of this cell surface glycoprotein remains unclear. In previous reports we demonstrated that PrP mediates cellular iron uptake and transport, and aggregation of PrP to the disease causing PrP-scrapie (PrP(Sc)) form results in imbalance of iron homeostasis in prion disease affected...
متن کاملGenetic human prion disease modelled in PrP transgenic Drosophila
Inherited human prion diseases, such as fatal familial insomnia (FFI) and familial Creutzfeldt-Jakob disease (fCJD), are associated with autosomal dominant mutations in the human prion protein gene PRNP and accumulation of PrPSc, an abnormal isomer of the normal host protein PrPC, in the brain of affected individuals. PrPSc is the principal component of the transmissible neurotoxic prion agent....
متن کاملAnti-Prion Drug mPPIg5 Inhibits PrP Conversion to PrP
Prion diseases, also known as transmissible spongiform encephalopathies, are a group of fatal neurodegenerative diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans. The ‘protein only hypothesis’ advocates that PrP, an abnormal isoform of the cellular protein PrP, is the main and possibly sole component of prion i...
متن کاملPrion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.
The 'protein only' hypothesis postulates that the prion, the agent causing transmissible spongiform encephalopathies, is PrP(Sc), an isoform of the host protein PrP(C). Protease treatment of prion preparations cleaves off approximately 60 N-terminal residues of PrP(Sc) but does not abrogate infectivity. Disruption of the PrP gene in the mouse abolishes susceptibility to scrapie and prion replic...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: British Medical Bulletin
سال: 2003
ISSN: 1471-8391,0007-1420
DOI: 10.1093/bmb/66.1.43