MIDBRAIN CATECHOLAMINERGIC NEURONS CO-EXPRESS α-SYNUCLEIN AND TAU IN PROGRESSIVE SUPRANUCLEAR PALSY

• Main Authors: María Elena eErro Aguirre (Author), María Victoria eZelaya (Author), Javier eSánchez Ruiz de Gordoa (Author), María Teresa eTuñón (Author), José Luis eLanciego (Author)
• Format: Book
• Published: Frontiers Media S.A., 2015-03-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Summary

Objective: To analyze the frequency and distribution of α-synuclein deposits in progressive supranuclear palsy (PSP).Methods: The brains of 25 cases of pathologically confirmed PSP were evaluated with immunohistochemistry for α-synuclein and tau. Multiple immunofluorescent stains were applied to analyze the expression of tau and α-synuclein aggregates in catecholaminergic neurons. Patients' clinical symptoms were retrospectively recorded. Results: Deposits α-synuclein in the form of typical Lewy bodies (LBs) were only found in two PSP cases (8%) that fulfilled the clinical subtype of PSP known as Richardson's syndrome (RS). LBs were present in the locus ceruleus, substantia nigra pars compacta, basal forebrain, amygdala and cingulated cortex in a distribution mimicking that of Parkinson's disease. Triple-immunolabeling revealed co-expression of α-synuclein and tau proteins in some tyrosine hydroxilase-positive neurons of the locus ceruleus and substantia nigra pars compacta.Conclusions: There is no apparent clinical correlation between the presence of LBs in PSP. Tau protein co-aggregate with α-synuclein in catecholaminergic neurons of PSP brains suggesting a synergistic interaction between the two proteins. This is in keeping with the current view of neurodegenerative disorders as 'misfolded protein diseases'.

Item Description

1662-5129
10.3389/fnana.2015.00025