Early destruction of the extracellular matrix around parvalbumin-immunoreactive interneurons in Creutzfeldt-Jakob disease1

Details

Serval ID
serval:BIB_DBD92C32C60A
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Early destruction of the extracellular matrix around parvalbumin-immunoreactive interneurons in Creutzfeldt-Jakob disease1
Journal
Neurobiology of Disease
Author(s)
Belichenko  P. V., Miklossy  J., Belser  B., Budka  H., Celio  M. R.
ISSN
0969-9961 (Print)
Publication state
Published
Issued date
1999
Volume
6
Number
4
Pages
269-279
Notes
PT - Journal Article PT - Research Support, Non-U.S. Gov't
Abstract
GABA-interneurons immunoreactive (IR) for the calcium-binding protein parvalbumin are lost during the early stages of Creutzfeldt-Jakob disease (CJD) and diminution in their number may partially account for the neurological disturbances manifested in patients suffering from this condition. The disease is characterized by a transformation of the prion protein, PrP(c)-a host-coded sialoglycoprotein-to its protease-resistant and putatively pathological form, PrP(CJD). And since this conversion is likely to take place at the cell surface, we were curious to know whether the "perineuronal net"-a characteristic accumulation of extracellular matrix in intimate contact with the surface of parvalbumin-IR neurons-is implicated in the early disappearance of the mantled cells. Using various lectins and antibodies as markers for the perineuronal net in brains of 21 CJD victims, we observed that this meshwork of extracellular matrix molecules is lost before the embraced parvalbumin-IR neurons themselves disappear. Hence, an interaction of PrP(c) and/or PrP(CJD) with components of the extracellular matrix around this subpopulation of nerve cells precipitates a sequence of as yet unknown events which culminates in the replacement of perineuronal nets by deposits of insoluble PrP(CJD). This change in the environment of the GABA-interneurons IR for parvalbumin may ultimately provoke their death
Keywords
Aged/Aged,80 and over/Calcium-Binding Protein,Vitamin D-Dependent/metabolism/Cell Count/Creutzfeldt-Jakob Syndrome/Pathology/Extracellular Matrix/Female/Histocytochemistry/Humans/Immunohistochemistry/Interneurons/Male/Middle Aged/Neurons/Parvalbumins/PrPSc Proteins/Tissue Distribution
Pubmed
Web of science
Create date
29/01/2008 18:36
Last modification date
20/08/2019 16:00
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