Related Experiment Video
Updated: Jul 10, 2026

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy
Published on: October 21, 2014
Vacuolar degeneration affecting brain macrophages/microglia in variant CJD: a report on two cases
1Department of Biological Sciences, Faculty of Science, The Open University, Milton Keynes, MK7 6AA, UK. p.rezaie@open.ac.uk
Abstract:
We present the neuropathology of two cases of variant Creutzfeldt-Jakob disease (vCJD) showing significant vacuolar degenerative alterations specifically affecting brain macrophages/microglia within the thalamus and, to a lesser extent, within the neocortical grey matter. Vacuolar degeneration in these cells was extensive, and likely to be associated with the development of a uniform sub-type of 'spongiform' vacuole seen in vCJD. The extensive morphological alterations described here closely resemble those very recently reported by Zucconi and colleagues, in response to experimental copper deficiency induced through dietary restriction, but could not be detected in cases of sporadic CJD examined. The significance of these novel findings are discussed in relation to copper homeostasis, loss of function of cellular prion protein and aberrant lysosomal catabolism within brain macrophages/microglia. This type of vacuolation may constitute a component of the overall profile of spongiform changes associated with vCJD.
More Related Videos
Related Concept Videos
Cerebral Edema ll: Pathophysiology
Alzheimer Disease ll: Pathophysiology
Lysosomal Hydrolases
Dementia l: Introduction
Alzheimer's Disease: Overview
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ and tau...
Cerebral Edema l: Introduction

