Prion plaques: molecular tumors. A hypothesis on the etiopathogenesis of prion diseases.
In spite of remarkable advances in the etiopathogenesis of spongiform encephalopathies in man and animals and the growing acceptance of the prion hypothesis, there is no explanation for the supposed 'autocatalytic' activity of this protein molecule. Our molecular tumor hypothesis proposes that the prion protein is a genotoxin which interacts directly or indirectly but specifically with its homologous cellular gene introducing mutations which lead to aberrant processing and accumulation of the protein. It is also speculated that this hypothesis would shed some light on other diseases not presently classified as prion diseases and in the process of ageing.
Ossa
J E
JE
Facultad de Medicina, Universidad de Antioquia, Medellin, Colombia.
Machado
G
G
Giraldo
M A
MA
McEwen
J G
JG
D000818:Animals; D002890:Chromosomes, Human, Pair 20; D004249:DNA Damage; D004268:DNA-Binding Proteins / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D008954:Models, Biological; D016296:Mutagenesis; D017096:Prion Diseases / Q000209:etiology* / Q000473:pathology; D011328:Prions / Q000235:genetics / Q000378:metabolism*
D004268:DNA-Binding Proteins; D011328:Prions