Prion plaques: Molecular tumors. A hypothesis on the etiopathogenesis of prion diseases

• Published in: Medical hypotheses Vol. 44; no. 2; pp. 124 - 126
• Main Authors: Ossa, J.E., Machado, G., Giraldo, M.A., McEwen, J.G.
• Format: Journal Article
• Language: English
• Published: Kidlington Elsevier Ltd 01-02-1995; Elsevier
• Subjects: Animals; Biological and medical sciences; Chromosomes, Human, Pair 20; DNA Damage; DNA-Binding Proteins - genetics; DNA-Binding Proteins - metabolism; Humans; Medical sciences; Mice; Mice, Transgenic; Models, Biological; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Mutagenesis; Neurology; Prion Diseases - etiology; Prion Diseases - pathology; Prions - genetics; Prions - metabolism; Human; Nervous system diseases; Animal; Central nervous system disease; Etiopathogenesis; Prion; Cerebral disorder
• ISSN: 0306-9877; 1532-2777
• DOI: 10.1016/0306-9877(95)90084-5

In spite of remarkable advances in the etiopathogeneis 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.