β-Cleavage of the prion protein in the human eye: Implications for the spread of infectious prions and human ocular disorders.

ABSTRACT: Recently, we reported β-cleavage of the prion protein (PrP^C) in human ocular tissues. Here, we explored whether this is unique to the human eye, and its functional implications. A comparison of the cleavage pattern of PrP^C in human ocular tissues with common nocturnal and diurnal animals revealed mainly β-cleavage in humans, and mostly full-length PrP^C in animal retinas. Soluble FL PrP^C and N-terminal fragment (N2) released from β-cleavage was observed in the aqueous and vitreous humor (AH & VH). Expression of human PrP^C in ARPE-19 cells, a human retinal pigmented epithelial cell line, also showed β-cleaved PrP^C. Surprisingly, β-cleavage was not altered by a variety of insults, including oxidative stress, suggesting a unique

SUBMITTER: Chaudhary S 

PROVIDER: S-EPMC8608713 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature