NR AXNG
AU Gmitterova,K; Heinemann,U.; Bodemer,M.; Krasnianski,A.; Meissner,B.; Zerr,I.
TI 14-3-3 Protein Cerebrospinal Fluid Levels in Sporadic Creutzfeldt-Jakob Disease Differ Across Molecular Subtypes
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Pathology and Pathogenesis P03.123
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB
Backround: The 14-3-3 protein is a physiological cellular protein expressed in various tissues, and its release to cerebrospinal fluid (CSF) reflects extensive neuronal damage as in Creutzfeldt-Jakob disease (CJD), but also in other neurological diseases. Many studies suggested that the 14-3-3 protein in CSF in the proper clinical context is a reliable diagnostic tool for sporadic CJD. However, the sensitivity varies across molecular CJD subtypes.
Objective: We determinated the level of the 14-3-3 protein in CSF from 70 sporadic CJD patients with distinct molecular subtypes by an enzyme-linked immunosorbent assay (ELISA) to prove whether different 14-3-3 levels might be helpful in the identification of different molecular subtypes.
Results: The 14-3-3 levels varied markedly across various molecular subtypes. The most elevated levels of 14-3-3 protein were observed in the frequently occurring and classical subtypes, whereas the levels were significantly lower in the subtypes with long disease duration and atypical clinical presentation. PRNP codon 129 genotype, PrPsc isotype, disease stage and clinical subtype influenced the 14-3-3 level and the test sensitivity. All heterozygous patients had 14-3-3 levels less than 3500 pg/ml irrespective of the PrPsc type and none of MV2 patients reached the level of 2300 pg/ml. At a cut-off of 2500 pg/ml, MM2 patients could be differentiated from MM1 patients. A tendency to lower 14-3-3 levels was observed in patients older than 60 years.
Conclusions: The 14-3-3 protein levels differ across molecular subtypes and in combination with other biomarkers might be used for their identification.
AD K Gmitterová, U. Heinemann, M. Bodemer, A. Krasnianski, B. Meissner, I. Zerr, Georg-August University, National TSE reference Center,Neurology, Germany
SP englisch
PO Schottland
EA pdf-Datei und Poster (Postertitel: CSF markers in the differentiation of molecular subtypes of sporadic CJD)