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The three glycotypes in the London classification system of sporadic Creutzfeldt-Jakob disease differ in disease duration

Citation

Ney, B and Eratne, D and Lewis, V and Ney, L and Li, Q-X and Stehmann, C and Collins, S and Velakoulis, D, The three glycotypes in the London classification system of sporadic Creutzfeldt-Jakob disease differ in disease duration, Molecular Neurobiology, 58, (8) pp. 3983-3991. ISSN 0893-7648 (2021) [Refereed Article]

Copyright Statement

The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021

DOI: doi:10.1007/s12035-021-02396-9

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of CJD and is believed to be caused by the misfolding and aggregation of endogenous prion protein. Several classification systems have been developed to correlate the molecular characteristics of these misfolded prions (PrPSc) to the heterogeneous clinical presentations of sCJD. A central component of these systems is glycotyping, which involves the interpretation of the results of western immunoblotting of the protease-resistant fragment of the misfolded prion protein (PrPres). The two main classification systems differ in their recognition of a unique banding pattern on electrophoretic gels correlating to a putative clinical subtype. The perpetuation of both classification systems within scientific literature is, in part, due to a paucity of high-level evidence that conclusively addresses the merit of recognising each unique banding pattern. Here, 110 post-mortem confirmed cases of sCJD collected at the Australian Creutzfeldt-Jakob Disease Registry (ANCJDR) between 1993 and 2018 were analysed and classified as per the London classification system. The data presented here demonstrated that sCJD cases with 'type 1' and 'type 2' PrPSc as defined by the London classification system differ in their disease duration. No other differences in clinical phenotype or biological characteristics were found to be statistically significant. These findings highlight the importance of sample size and replicability in analyses of this rare disease process. Recognising these glycotypes as phenotypically distinct may represent 'best practice' in the collection and processing of sCJD samples within international registries for research purposes.

Item Details

Item Type:Refereed Article
Keywords:glycotype, sporadic Creutzfeldt-Jakob disease, SCJD
Research Division:Biomedical and Clinical Sciences
Research Group:Neurosciences
Research Field:Neurology and neuromuscular diseases
Objective Division:Health
Objective Group:Clinical health
Objective Field:Diagnosis of human diseases and conditions
UTAS Author:Ney, L (Mr Luke Ney)
ID Code:144199
Year Published:2021
Deposited By:Psychology
Deposited On:2021-04-27
Last Modified:2022-01-17
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