Penetration of Infectious Prion Protein in the Intestine During the Lactation Period

Ryuta      Uraki; Akikazu      Sakudo; Yasuhisa      Ano; Juri      Kono; Masayoshi      Yukawa; Gianluigi      Zanusso; Antonio      Toniolo; Takashi      Onodera

Abstract

Prion diseases are fatal neurodegenerative zoonotic foodborne disorders, which are caused by an abnormal isoform of prion protein (PrPSc) derived from the cellular isoform of prion protein (PrPC). According to epidemiological surveillance and in vivo experiments, exposure to the PrPSc during the weaning period is fraught with risk, suggesting that, during development, the intestinal defenses and the immune system are involved in PrPSc infection susceptibility. Although it remains unclear how PrPSc passes through the natural biological barriers during its invasion of intestinal cells, the 37 kDa/67 kDa laminin receptor is suspected to be one of the receptors involved in PrPSc-incorporation. In addition, we have recently shown that the neonatal Fc receptor (nFcR), which contributes to the uptake of maternal antibodies into the intestine, may play an important role in PrPSc incorporation. In this review, recent studies on PrPSc uptake and models of PrPSc incorporation into the intestine via the laminin and Fc receptors are described.

Keywords: Prion disease, PrPSc uptake, Fc receptor, neurodegenerative diseases, follicular dendritic cells, immunoglobulins, epithelial barriers, immunohistochemistry, laminin receptor precursors