Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.

Mechanisms of prion-induced neurodegeneration.

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative disorders characterised by long incubation period, short clinical duration, and transmissibility to susceptible species. Neuronal loss, spongiform changes, gliosis and the accumulation in the brain of the misfolded version of a membrane-bound cellular prion protein (PrP(C)), termed PrP(TSE), are dia...

Prion infection impairs the cellular response to oxidative stress.

The molecular mechanism of neurodegeneration in transmissible spongiform encephalopathies remains uncertain. In this study, it was demonstrated that prion-infected hypothalamic neuronal GT1 cells displayed a higher sensitivity to induced oxidative stress over noninfected cells. In addition, the infected cells presented an increased lipid peroxidation and signs of apoptosis associated with a dra...

Mechanisms of Cell Death in the Transmissible Spongiform Encephalopathies

Mayhem of the multiple mechanisms: modelling neurodegeneration in prion disease.

This review examines recent attempts to advance the understanding of the mechanism by which neurones die in prion disease. Prion diseases or transmissible spongiform encephalopathies are characterized by the conversion of a normal glycoprotein, the prion protein, to a protease-resistant form that is suggested to be both the infectious agent and the cause of the rapid neurodegeneration in the di...

Transmissible spongiform encephalopathies.

Vet Med Today: Zoonosis Update 1705 T spongiform encephalopathies affect humans and other animals. Clinically, the diseases involve severe, progressive neurodegeneration with an invariably fatal outcome. The TSEs are linked by the unusual nature of their causative agent, which is believed to be a transmissible protein devoid of nucleic acid. This protein, known as a prion (a pseudoabbreviation ...