NR AOFR
AU Dimcheff,D.E.; Portis,J.L.; Caughey,B.W.
TI Prion proteins meet protein quality control
QU Trends in Cell Biology 2003 Jul; 13(7): 337-40
PT journal article; review; review, tutorial
AB Recent work on transmissible spongiform encephalopathies (TSEs) suggests a role for protein quality-control mechanisms in both prion protein aggregation and pathogenesis. Cytosolic accumulation of prion protein seems to be neurotoxic and might occur when proteasome function is compromised and quality control is overwhelmed. These findings are discussed in the light of other studies linking proteasome inhibition and neurodegeneration.
ZR 17
MH Animal; Cysteine Endopeptidases/metabolism; Cytosol/metabolism; Human; Multienzyme Complexes/metabolism; Nerve Degeneration/metabolism/physiopathology; Neurons/*metabolism/pathology; PrPc Proteins/*metabolism; PrPsc Proteins/*metabolism; Prion Diseases/*metabolism/physiopathology; *Protein Folding; Protein Transport/physiology; Support, U.S. Gov't, P.H.S.
AD Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases/NIH, Hamilton, MT 59840, USA. ddimcheff@nih.gov
SP englisch
PO England