NR AICY
AU Mayer,R.J.; Landon,M.; Laszlo,L.; Lennox,G.; Lowe,J.
TI Protein processing in lysosomes: the new therapeutic target in neurodegenerative disease.
QU Lancet 1992 Jul 18; 340(8812): 156-9
PT journal article; review; review, tutorial
AB A little recognised feature of neurons is their large complement of lysosomes. Studies of the accumulation of the abnormal isoform of the prion protein (PrPsc) in the prion encephalopathies and the formation of beta/A4 protein from its precursor in Alzheimer's disease suggest that generation of these key proteins takes place in lysosome-related organelles. The release of hydrolytic enzymes from lysosomes may be a primary cause of neuronal damage. Although molecular genetic approaches have identified protein mutations central to the main neurodegenerative disease, cell biological observations are now beginning to unravel the intracellular pathways involved in the molecular pathogenesis of neurodegeneration: as a result, it is now appropriate to consider therapeutic manipulation of the lysosomal system as an approach to treatment.
ZR 36
MH Animal; Brain/metabolism/pathology; Human; Lysosomes/*metabolism; Nervous System Diseases/*metabolism; Neurobiology; PrPsc Proteins; Prions/*metabolism; Support, Non-U.S. Gov't; Ubiquitins/*physiology
AD Department of Biochemistry, University of Nottingham Medical School, Queen's Medical Centre, UK
SP englisch
PO England