Cerebral glycosidases in experimental Creutzfeldt-Jakob disease.

Abstract:

:In Creutzfeldt-Jakob disease (CJD), there are prominent ultrastructural alterations of the plasma membrane, which contains many glycolipids and glycoproteins. Glycosidases can degrade glycolipids and glycoproteins. Gangliosides, a subset of glycolipids, are decreased in amount at the terminal stages of CJD, and CJD infectivity is closely associated with membrane rich fractions. We therefore studied 10 glycosidases, and found a statistically significant increase in beta-xylosidase, beta-glucuronidase, N-acetyl-beta-D-glucosaminidase and N-acetyl-beta-D-galactosaminidase activities in CJD. In contrast, alpha-glucosidase, beta-glucosidase, alpha-galactosidase, alpha-mannosidase, alpha-fucosidase, and beta-galactosidase were not significantly changed. The above results are consistent with degenerative membrane changes observed morphologically, and with increased degradation of sugar residues on lipids and/or proteins. These changes may be effected by the accumulation of the CJD agent in cell membranes. We suggest that the higher activities of these enzymes in CJD may be partially responsible for some of the structural and biochemical alterations in CJD infected brains.

journal_name

J Neurol Sci

authors

Kim JH,Manuelidis L,Manuelidis EE

doi

10.1016/0022-510x(88)90178-5

subject

Has Abstract

pub_date

1988-03-01 00:00:00

pages

95-100

issue

1

eissn

0022-510X

issn

1878-5883

pii

0022-510X(88)90178-5

journal_volume

84

pub_type

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