2019 Volume 70 Pages 13-17
The human epidermal growth factor receptor family consists of four members that belong to the ErbB proteins (ErbB1-4). Neuregulin-1 (NRG1)/ErbB signaling regulates brain development, synaptic plasticity and neuronal survival. Abnormalities in this signaling have been implicated in the etiology or development of neurodegenerative diseases such as Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis. So, we aimed to investigate whether the expression of NRG1 or ErbB proteins are altered in progressive supranuclear palsy (PSP). Whereas C-terminal ErbB4 immunoreactivity was partially but distinctly present in the cytoplasm and/or in the nucleus of neurons in control patients, it was rarely observed in the neuronal nuclei in PSP patients. In contrast, neurofibrillary tangles, coiled bodies, and threads were robustly immunoreactive for C-terminal ErbB4 in PSP. Our present results suggest that disturbed NRG1/ErbB4 signaling could be an important event in the pathogenesis of PSP.
