Influence of polymorphisms in the prion protein gene on the pathogenesis and neuropathological phenotype of sheep scrapie after oral infection

Journal of Comparative Pathology
L GonzálezM Jeffrey

Abstract

The prion protein gene (Prnp) plays a crucial role in the susceptibility of sheep to scrapie in terms of attack rate and/or incubation period. However, the influence of Prnp on the pathogenesis of the disease, specifically the involvement of tissues of the lymphoreticular system (LRS), pathways of neuroinvasion and neuropathological phenotypes, remains controversial. This study reports the onset and progression of disease-associated prion protein (PrP(d)) accumulation in the LRS and nervous tissues of sheep of six different Prnp genotypes infected by oral administration of the same mixed scrapie brain homogenate. Sheep homozygous for glutamine (Q) at codon 171 of PrP, with either valine (V) or alanine (A) at codon 136 (i.e. VRQ/VRQ, VRQ/ARQ and ARQ/ARQ), showed early and consistent PrP(d) accumulation in LRS tissues of the pharynx and gut. In contrast, LRS involvement was minimal, inconsistent and occurred late in the incubation period in sheep heterozygous for arginine (R) at codon 171 (i.e. VRQ/ARR and ARQ/ARR). Despite this difference, all five groups were susceptible to infection and developed clinical disease, albeit with significantly different incubation periods (shortest in VRQ/VRQ and longest in ARQ/ARR sheep). The rem...Continue Reading

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Citations

May 23, 2015·Tropical Animal Health and Production·Caroline Pinto de AndradeDavid Driemeier
Mar 12, 2016·PloS One·Gillian McGovernLorenzo González
Oct 2, 2015·Journal of Virology·Camilo Duque VelásquezDebbie McKenzie
Sep 19, 2017·The Journal of General Virology·Rosa BoleaJuan José Badiola
Sep 12, 2018·Veterinary Pathology·Justin J Greenlee
Aug 10, 2017·EFSA Journal·UNKNOWN EFSA Panel on Biological Hazards (BIOHAZ)Emma Snary
Feb 8, 2017·Food Safety·John Spiropoulos, Marion M Simmons
Dec 1, 2020·Neuropathology and Applied Neurobiology·Juan Carlos EspinosaJuan María Torres

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