Transmissible Spongiform Encephalopathies Flashcards
What is TSE in sheep?
Scrapie
What is TSE in cattle?
BSE
What is TSE in deer?
CWD
chronic wasting disease
What is TSE in humans?
variant Creutzfelt-Jakob disease
What causes TSE?
*PRP - protease resistant protein
*PRPc + PRPsc = identical amino acid sequence but different tertiary structure
*PRPsc converts PRPc into itself - PRPsc is partially resistant to digestion + accumulates = disease + death
What are the characteristics of TSE?
- Long incubation period
- Progressive and invariably fatal
- Holes in brain give spongiform texture
- No signs of fever
- No signs of inflammation
- No antibody response
- No signs of a pathogen with a genome
- No sign of infection
What are the clinical signs of scrapie?
*Ataxia
*Tremors
*Rubbing / scratching
*Loss of condition
*Death in 1-3 months
What is the incubation period of scrapie?
2-5 years
How can sheep catch scrapie?
*Vertical
*Pseudo-vertical
*Horizontal
*Contaminated land
*Iatrogenic
How does Vertical transmission of scrapie occur?
Mother to offspring
*in utero
How does Pseudo-vertical transmission of scrapie occur?
Parent to offspring
*Lambs infected at birth - from infected birth tissues
How does horizontal transmission of scrapie occur?
Adult to adult / lamb
*Infected sheep spread infection to naive adult sheep in same flock
How does contaminated land transmission of scrapie occur?
Sheep catch scrapie on land where infected sheep used to live - decades before
How does Iatrogenic transmission of scrapie occur?
Needle contamination
What are the important PrP alleles in
UK sheep?
What codons are disease linked with scrapie?
*ARR - resistance
*AHQ - resistance with VRQ
*ARQ - susceptibility
*ARH - susceptibility with VRQ
*VRQ - susceptibility
Codons = 136, 154 + 171
(ARR = Alanine coded at 136, aRginine coded at 154 + 171)