prion and prion-like diseases

Question 1

what are the diseases for the sporadic, genetic and acquired forms of prion diseases

Answer 1

sporadic : Creutzfeldt-Jacob (CJD)
genetic : fCJD, Gerstmann-syndrome (GSS), fatal insomnia (fFI)
acquired : Kuru (eating brain of dead people), Iatrogeneis CJD, vCJD

Question 2

what is TSE

Answer 2

TSE: Transmissible Spongioform Encephalopathy, which is a source of acquired prion disease

this can come from the food industry, organ transplant, blood, etc

Question 3

wat are prion diseases

Answer 3

fatal diseases caused by an infectious agent known as a prion, which is derived from a misfolded version of a normal host protein known as prion protein.

Question 4

describe the prion pathology

Answer 4

1. holes in tissue
2. spongiformic changes in grey matter
3. large vacuoles

Question 5

what are prions

Answer 5

infectious particles, which transmit their misfolded conformation to healthy proteins.

Question 6

which protective allele is there for prion diseases

Answer 6

the G127V (found in the fore people after Kuru epidemic)

Question 7

What did the long-term graft reveal about the viability in PD

Answer 7

The embryonal graft contained Lewy bodies, which indicates that the graft cells have obtained the disease from it’s host

Question 8

does a-synuclein have a prion like spreading

Answer 8

yes, intracerebral inoculation of a-syn has proven to spread to the ipsilateral and then contralateral brain areas

Question 9

what are potential mechanisms for trans-cellular propagation of protein misfolding

Answer 9

1. cell dies –> aggregate uptake by viable cells
2. exocytosis
3. synaptic release