title: Tau and 14-3-3 of genetic and sporadic Creutzfeldt-Jakob disease patients in Israel.
abstract: One of the largest clusters of genetic Creutzfeldt-Jakob disease (gCJD) is found among Jews of Libyan origin in Israel and is linked to the E200K mutation in PRNP (gCJDE200K). The aim of this study was to compare the levels of cerebrospinal fluid (CSF) biomarkers, Tau and 14-3-3 proteins, between gCJDE200K patients, sporadic CJD (sCJD) patients and non-CJD controls in Israel between the years 1996-2006. The levels of Tau and 14-3-3 proteins in CSF were measured by ELISA and immunoblotting, respectively. CSF Tau levels were similar in gCJDE200K and sCJD, both were significantly higher than in controls [1,107 ± 470 pg/ml [33/46 (72%)] of the cases >1,000 pg/ml, 1,280 ± 580 pg/ml [25/30 (83.3%)], and 354 ± 338 pg/ml [17/243 (6.9%)], respectively, p < 0.001]. 14-3-3 was detected in CSF of 41/53 (77%) of each gCJDE200K and sCJD patients tested, but only in 70/417 (16.8%) of controls (p < 0.001). An inverse correlation was found between disease duration and Tau levels in both gCJDE200K and sCJD (r = -0.464 and r = -0.284). No difference was found in Tau or 14-3-3 between the various codon 129 genotypes. We conclude that CSF biomarkers, Tau and 14-3-3, may be used in the diagnosis in both patients' populations, presenting a similar sensitivity yet Tau assay having higher specificity.
authors.affiliation: Departments of Neurology, Hadassah University Hospital, Jerusalem, Israel. meiner@hadassah.org.il
authors.forename: Zeev
authors.initials: Z
authors.lastname: Meiner
authors.forename: Esther
authors.initials: E
authors.lastname: Kahana
authors.forename: Fanny
authors.initials: F
authors.lastname: Baitcher
authors.forename: Amos D
authors.initials: AD
authors.lastname: Korczyn
authors.forename: Joab
authors.initials: J
authors.lastname: Chapman
authors.forename: Oren S
authors.initials: OS
authors.lastname: Cohen
authors.forename: Ron
authors.initials: R
authors.lastname: Milo
authors.forename: Judith
authors.initials: J
authors.lastname: Aharon-Perez
authors.forename: Oded
authors.initials: O
authors.lastname: Abramsky
authors.forename: Ruth
authors.initials: R
authors.lastname: Gabizon
authors.forename: Hanna
authors.initials: H
authors.lastname: Rosenmann
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid* / Q000235:genetics; D000368:Aged; D015415:Biomarkers / Q000134:cerebrospinal fluid; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid* / Q000235:genetics; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D015151:Immunoblotting; D007557:Israel; D008297:Male; D008875:Middle Aged; D009154:Mutation; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics; D012680:Sensitivity and Specificity; D016875:tau Proteins / Q000134:cerebrospinal fluid* / Q000235:genetics
chemical_list: D048948:14-3-3 Proteins; D015415:Biomarkers; C512656:PRNP protein, human; D000072002:Prion Proteins; D011328:Prions; D016875:tau Proteins
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