M19 - Prions (sterilisation) Flashcards
Name prion based diseases.
- Scrapie (BSE of cattle)
- Creutzfeldt- Jakob disease
(sporadic / inherited) - Gerstmann- straussler- Schenker
-Kuru
-New variant CJD
what is the most common type of Creutzfeldt- Jacob disease (CJD)
Sporadic
who is most effective by sporadic CJD?
patient over 60 years old (elderly)
what happens in sporadic CJD?
rapid dementia to death (4.5 months)
where are the highest levels of prions in sporadic CJD?
in CNS , also retina and sensory ganglia, but not in blood
what is the other type of CJD which effects younger patient in their 30s?
Variant
what is variant CJD?
fatal Human neurodegenerative condition classified as transmissible spongiform encephalopathy
what is the risk of variant CJD?
Risk of person to person spread by contaminated surgical- instruments that have not been cleaned, disinfected
what prion protein is found in vCJD?
PrPsc
what is important in CJD?
PRNP codon 129 of Prion Protein
what Amino acids are found are position 129?
methionine or valine
What are the different genotypes of vCJD
- Homozygous MM
- Homozygous VV
- Heterozygous MV
what is the genotype of 177 of 178 cases of vCJD?
MM
what gene appears significant in CJD?
PrP gene
-amino acid variants
what are the symptoms of vCJD?
-Average age 28 (65) -progressive dementia, sensory symptoms, unsteadiness,
involuntary movements, immobile & mute
death <14 mths
what happens in the brain in vCJD?
- Brain vacuolation, neural apoptosis
- Accumulate misfolded protease- resistant prion protein
- Protease Resistant Prion protein PrPSc