Cellular prion protein is present in mitochondria of healthy mice.

ABSTRACT: Cellular prion protein (PrP^C) is a mammalian glycoprotein which is usually found anchored to the plasma membrane via a glycophosphatidylinositol (GPI) anchor. PrP^C misfolds to a pathogenic isoform PrP^Sc, the causative agent of neurodegenerative prion diseases. The precise function of PrP^C remains elusive but may depend upon its cellular localization. Here we show that PrP^C is present in brain mitochondria from 6-12 week old wild-type and transgenic mice in the absence of disease. Mitochondrial PrP^C was fully processed with mature N-linked glycans and did not require the GPI anchor for localization. Protease treatment of purified mitochondria suggested that mitochondrial PrP^C exists as a transmembrane isoform with the C-terminus facing the mitochondrial matrix and the N-terminus facing the intermembrane space. Taken together, our data suggest that PrP^C can be found in mitochondria in the absence of disease, old age, mutation, or overexpression and that PrP^C may affect mitochondrial function.

SUBMITTER: Faris R 

PROVIDER: S-EPMC5288712 | biostudies-literature | 2017 Feb

REPOSITORIES: biostudies-literature