Handout 9 - Prions

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3/26/2006 Replication This causes a conformational change in the PrP 27-30 protein from an α-helix to a β-sheet. This conformational change in the PrP 27-30 protein has three effects: 1. It imparts to the PrP 27-30 protein the ability to induce the same α -helix to β -sheet conformation in the PrP 33-35 protein. This is a permanent conformational change. It thus induces its own "replication." 2. The β-sheet-forming peptides aggregate to form amyloid fibrils. 3. The amyloid fibrils kill thalamus neurons through apoptosis. Pathologies induced by prions All diseases known to be of prion etiology, in animals and humans, are neurodegenerative diseases. In the human this includes: •Creutzfeldt-Jakob disease (CJD) •Fatal Familial Insomnia •Gerstmann-Straussler syndrome •Kuru 3/26/2006
Pathologies induced by prions The pathological and clinical signs of these diseases suggest that they are closely related. In fact they may be variants of the same disorder. All pathological features are confined to the central nervous system. The prion protein accumulates selectively and abnormally in CNS nerve cells during the course of the disease. 3/26/2006 Pathologies induced by prions PrP 27-30 accumulates within the neuronal system where it causes: 1. Astrocyte gliosis (an increase in the number of astrocytes). 2. Depletion of dendritic spines in neurons. 3. Formation of numerous vacuoles in the cerebellar cortex (spongiform encephalopathy). 3/26/2006
Page 1 and 2: 3/26/2006 3/26/2006 PRIONS Definiti
Page 3 and 4: Humans diseases • The modified fo
Page 5: 3/26/2006 Replication In normal cel
Page 9 and 10: Pathologies induced by prions These
Page 11 and 12: 3/26/2006 Prion Diseases Prion dise
Page 13 and 14: Humans are also susceptible to seve
Page 15 and 16: Humans might be infected by prions
Page 17 and 18: 2. PrPsc is associated with scrapie
Page 19 and 20: 4. Mutated gene can cause susceptib
Page 21 and 22: 3/26/2006 Evidence against the prio
Page 23 and 24: Treatment •PrPc overexpression fa

Handout 9 - Prions

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