Goats without Prion Protein Display Enhanced Proinflammatory Pulmonary Signaling and Extracellular Matrix Remodeling upon Systemic Lipopolysaccharide Challenge.

ABSTRACT: A naturally occurring mutation in the PRNP gene of Norwegian dairy goats terminates synthesis of the cellular prion protein (PrP^C), rendering homozygous goats (PRNP^Ter/Ter) devoid of the protein. Although PrP^C has been extensively studied, particularly in the central nervous system, the biological role of PrP^C remains incompletely understood. Here, we examined whether loss of PrP^C affects the initial stage of lipopolysaccharide (LPS)-induced acute lung injury (ALI). Acute pulmonary inflammation was induced by intravenous injection of LPS (Escherichia coli O26:B6) in 16 goats (8 PRNP^Ter/Ter and 8 PRNP^+/+). A control group of 10 goats (5 PRNP^Ter/Ter and 5 PRNP<

SUBMITTER: Salvesen O 

PROVIDER: S-EPMC5723645 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature