The Elusive Prions and CJD: possible to treat?
Introduction to Prion Disease
Prion disease is an infectious neurodegenerative disease caused by a naturally occurring protein (PrPC) misfolding into an aberrant form (PrPSC) which aggregates and destroys neuronal tissue. PrPSC was originally posited by Stanley Prusiner to be the sole causative moiety in prion disease (Prion Theory), however there is some evidence to suggest that unknown toxic intermediates, co-factors, and certain nucleic acids may have roles to play in its pathogenesis. In humans the diseases are named Creutzfeldt-Jakob Disease (CJD), GerstmannSträussler-Scheinker (GSS) syndrome, Fatal Familial Insomnia (FFI) and kuru. These conditions differ in aetiology, the cause of PrP misfolding, but all are inexorably fatal. As of yet there is no effective cure. The incubation times of prion diseases are usually long (years), but once clinically apparent are rapidly progressive.
Resistance to treatment & current
The disease-causing moiety, whether protein-only or
not, is remarkably resistant to a myriad of physical ,
chemical and biological treatments and retains
• Irradiation with UV light
• Intense heat of 132*C for 30 minutes
Research Proposal: Lichens – natural born prion
Lichens are composite symbiotic organisms consisting of a fungus and a photosynthetic organism (usually algae or a cyanobacteria).
Current approaches to therapy :
Image showing the spongiform changes found in
brains of prion disease-affected individuals.
Sporadic CJD (sCJD) – the seemingly ‘random’ conversion of normal
Inhibition of PrPC synthesis (prion replication
requires the presence of normal cellular PrP as a
• Stabilising PrPC conformation
• Enhancing PrPSC clearance
• Preventing PrPSC replication
PrPC to pathogenic PrPSC, showing a mean clinical presentation age of 55-60 years with a short duration of...
References: Concepts. Cell, 2004. Vol. 116. 313-327.
Protein by a Serine Protease from Lichens. PloS One, 2011. Vol 6 Issue 5.
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