Protective effect of the T112 PrP variant in sheep challenged with bovine spongiform encephalopathy.
Sheep with an ARQ/ARQ PRNP genotype at codon positions 136/154/171 are highly susceptible to experimental infection with bovine spongiform encephalopathy (BSE). However, a number of sheep challenged orally or intracerebrally with BSE were clinically asymptomatic and found to survive or were diagnosed as BSE-negative when culled. Sequencing of the full PRNP gene open reading frame of BSE-susceptible and -resistant sheep indicated that, in the majority of Suffolk sheep, resistance was associated with an M112T PRNP variant (TARQ allele). A high proportion (47 of 49; 96%) of BSE-challenged wild-type (MARQ/MARQ) Suffolk sheep were BSE-infected, whereas none of the 20 sheep with at least one TARQ allele succumbed to BSE. Thirteen TARQ-carrying sheep challenged with BSE are still alive and some have survival periods equivalent to, or greater than, reported incubation periods of BSE in ARR/ARR and VRQ/VRQ sheep.
Saunders
G C
GC
Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency (VLA Weybridge), New Haw, Addlestone, Surrey KT15 3NB, UK.
Lantier
I
I
INRA, UR1282, Infectiologie Animale et Santé Publique, F-37380 Nouzilly, France.
Cawthraw
S
S
Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency (VLA Weybridge), New Haw, Addlestone, Surrey KT15 3NB, UK.
Berthon
P
P
INRA, UR1282, Infectiologie Animale et Santé Publique, F-37380 Nouzilly, France.
Moore
S J
SJ
Pathology Department, VLA Weybridge, New Haw, Addlestone, Surrey KT15 3NB, UK.
Arnold
M E
ME
CERA, VLA Weybridge, New Haw, Addlestone, Surrey KT15 3NB, UK.
Windl
O
O
Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency (VLA Weybridge), New Haw, Addlestone, Surrey KT15 3NB, UK.
Simmons
M M
MM
Pathology Department, VLA Weybridge, New Haw, Addlestone, Surrey KT15 3NB, UK.
Andréoletti
O
O
UMR INRA-ENVT, Interactions Hôtes-Agents Pathogènes, Ecole Vétérinaire de Toulouse, F-310761 Toulouse, France.
Bellworthy
S
S
Pathology Department, VLA Weybridge, New Haw, Addlestone, Surrey KT15 3NB, UK.
Lantier
F
F
INRA, UR1282, Infectiologie Animale et Santé Publique, F-37380 Nouzilly, France.
D000818:Animals; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000235:genetics / Q000276:immunology / Q000635:transmission*; D020022:Genetic Predisposition to Disease; D005838:Genotype; D011328:Prions / Q000276:immunology*; D012756:Sheep; D012757:Sheep Diseases / Q000235:genetics / Q000276:immunology*
D011328:Prions