TY - JOUR
T1 - The ε isoform of 14-3-3 protein is a component of the prion protein amyloid deposits of Gerstmann-Sträussler-Scheinker disease
AU - Di Fede, Giuseppe
AU - Giaccone, Giorgio
AU - Limido, Lucia
AU - Mangieri, Michela
AU - Suardi, Silvia
AU - Puoti, Gianfranco
AU - Morbin, Michela
AU - Mazzoleni, Giulia
AU - Ghetti, Bernardino
AU - Tagliavini, Fabrizio
PY - 2007/2
Y1 - 2007/2
N2 - The 14-3-3 proteins are highly conserved, ubiquitous molecules involved in a variety of biologic events, such as transduction pathway modulation, cell cycle control, and apoptosis. Seven isoforms have been identified that are abundant in the brain, preferentially localized in neurons. Remarkable increases in 14-3-3 are seen in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease (CJD), and it has been found in pathologic inclusions of several neurodegenerative diseases. Moreover, the ζ isoform has been detected in prion protein (PrP) amyloid deposits of CJD patients. To further investigate the cerebral distribution of 14-3-3 in prion-related encephalopathies, we carried out an immunohistochemical and biochemical analysis of brain tissue from patients with Gerstmann-Sträussler-Scheinker disease (GSS) and sporadic, familial and acquired forms of CJD, using specific antibodies against the seven 14-3-3 isoforms. The study showed a strong immunoreactivity of PrP amyloid plaques of GSS patients for the 14-3-3 ε isoform, but not for the other isoforms. The ε isoform of 14-3-3 was not found in PrP deposits of CJD. These results indicate that the ε isoform of 14-3-3 is a component of PrP amyloid deposits of GSS and suggest that this is the sole 14-3-3 isoform specifically involved in the neuropathologic changes associated with this disorder.
AB - The 14-3-3 proteins are highly conserved, ubiquitous molecules involved in a variety of biologic events, such as transduction pathway modulation, cell cycle control, and apoptosis. Seven isoforms have been identified that are abundant in the brain, preferentially localized in neurons. Remarkable increases in 14-3-3 are seen in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease (CJD), and it has been found in pathologic inclusions of several neurodegenerative diseases. Moreover, the ζ isoform has been detected in prion protein (PrP) amyloid deposits of CJD patients. To further investigate the cerebral distribution of 14-3-3 in prion-related encephalopathies, we carried out an immunohistochemical and biochemical analysis of brain tissue from patients with Gerstmann-Sträussler-Scheinker disease (GSS) and sporadic, familial and acquired forms of CJD, using specific antibodies against the seven 14-3-3 isoforms. The study showed a strong immunoreactivity of PrP amyloid plaques of GSS patients for the 14-3-3 ε isoform, but not for the other isoforms. The ε isoform of 14-3-3 was not found in PrP deposits of CJD. These results indicate that the ε isoform of 14-3-3 is a component of PrP amyloid deposits of GSS and suggest that this is the sole 14-3-3 isoform specifically involved in the neuropathologic changes associated with this disorder.
KW - 14-3-3
KW - Amyloid
KW - Creutzfeldt-Jakob disease
KW - Gerstmann-Sträussler- Scheinker disease
KW - Prion protein
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U2 - 10.1097/nen.0b013e3180302060
DO - 10.1097/nen.0b013e3180302060
M3 - Article
C2 - 17278997
AN - SCOPUS:33846811283
VL - 66
SP - 124
EP - 130
JO - American Journal of Psychotherapy
JF - American Journal of Psychotherapy
SN - 0002-9564
IS - 2
ER -