Neuronal autophagy in experimental Creutzfeldt-Jakob's disease.

Abstract:

:We report an experimental model of Creutzfeldt-Jakob's disease (CJD) in mice leading to the formation of giant autophagic vacuoles (AV) in neurons of the cerebral cortex. These AV appear at the end of the incubation period (4-6 months postinoculation), together with spongy changes and clinical symptoms. Autophagy, a process of intracellular digestion of cell constituents by the lysosomal compartment, is known in many cell types, where it plays a role both in the physiological turnover and in pathological processes and is involved in protein metabolism. The same also occurs in neurons. Here autophagy is known to occur in the normal state and leads to residual bodies called lipofuscin granules. An increase in lipofuscin is known to occur in human and experimental CJD. Therefore, an increase in autophagy and in AV can be expected. In our experimental model, the activation of neuronal autophagy may be related to an alteration in neuronal protein metabolism.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Boellaard JW,Schlote W,Tateishi J

doi

10.1007/BF00688178

subject

Has Abstract

pub_date

1989-01-01 00:00:00

pages

410-8

issue

4

eissn

0001-6322

issn

1432-0533

journal_volume

78

pub_type

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