Neurotoxicity of a prion protein fragment
Article Abstract:
A study to test the neurotoxicity of a protein fragment from the human cellular prion protein (PrP) is presented. PrP is a sialoglycoprotein expressed predominantly in neurons and which is converted into a protease-resistant analogue in genetic neurodegenerative diseases of some vertebrates. The results showed that chronic exposure to micromolar concentrations of a peptide corresponding to PrP residues 106-126 caused neuronal death in primary rat hippocampal cultures. This suggests that cerebral accumulation of PrP and its degradation products have a role in PrP-related neurodegenerative diseases.
Publication Name: Nature
Subject: Zoology and wildlife conservation
ISSN: 0028-0836
Year: 1993
User Contributions:
Comment about this article or add new information about this topic:
The cellular prion protein binds copper in vivo
Article Abstract:
The precursor to the pathogenic protease-resistant forms (PrPSc) thought to cause scrapie, bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob disease, is the normal cellular form of prion protein (PrPC). It is shown that the amino-terminal domain of PrPC has five to six sites binding copper presented as a glycine chelate. Binding occurs with positive cooperativity at neutral pH, compatible with estimates for extracellular, labile copper. It is suggested that PrPC can exist in a Cu-metalloprotein form in vivo.
Publication Name: Nature
Subject: Zoology and wildlife conservation
ISSN: 0028-0836
Year: 1997
User Contributions:
Comment about this article or add new information about this topic:
Role of microglia and host prion protein in neurotoxicity of a prion protein fragment
Article Abstract:
The prion protein is a conjugated protein which has a carbohydrate as its nonprotein group and is usually found in neuroglia. It is associated with bovine spongiform encephalopathy, scrapie and Creutzfeldt-Jakob disease. Prior diseases are distinguished by neuronal degeneration, gliosis and accumulation of prion protein. Mice without prion protein are not infected by scrapie.
Publication Name: Nature
Subject: Zoology and wildlife conservation
ISSN: 0028-0836
Year: 1996
User Contributions:
Comment about this article or add new information about this topic:
- Abstracts: Notch signalling regulates stem cell numbers in vitro and in vivo. Caught in time
- Abstracts: Evaluation of age determination techniques for gray wolves. Effects of recreation and hunting on flushing distance of Capercaillie
- Abstracts: Botulinum neurotoxin B recognizes its protein receptor with high affinity and specificity. Substrate recognition strategy for botulinum neurotoxin serotype A
- Abstracts: Crystal structure of a small G protein in complex with the GTPase-activating protein rhoGAP
- Abstracts: Neophilia and exploration in juvenile common ravens, Corvus corax. Raven roosts are mobile information centres