Prion Diseases

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Total cards: 24•Content version: v4•Public deck•Updated: 8/1/2026

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Front (Question)

What are abnormal prions

Back (Answer)

Abnormal prions are infectious misfolded proteins that can trigger abnormal folding of other prions

Front (Question)

Which level of protein structure do prions affect

Back (Answer)

Secondary structure

Front (Question)

What is the largest class of protein secondary structures

Back (Answer)

alpha helices

Front (Question)

Function of alpha helices

Back (Answer)

They have a major role in mediating protein-protein interactions (allows hydrogen bonding)

Front (Question)

What does CJD stand for

Back (Answer)

Creutzfeldt-Jakob disease

Front (Question)

what does vCJD stand for

Back (Answer)

Variable Creutzfeldt-Jakob Disease

Front (Question)

What does BSE stand for

Back (Answer)

Bovine Spongiform Encephalopathy

Front (Question)

Description of CJD

Back (Answer)

A transmissible subacute spongiform encephalopathy

Front (Question)

Subacute meaning

Back (Answer)

There is a very long incubation time (long time between getting the disease and showing symptoms)

#10

Front (Question)

What does encephalopathy mean

Back (Answer)

Brain disease

#11

Front (Question)

What does spongiform mean

Back (Answer)

Brain tissue turns into a sponge like pattern

#12

Front (Question)

CJD aetiology (3)

Back (Answer)

1.Sporadic -> (majority of cases), hypothesised to occur as a result of aging cellular machinery, sporadic mutation of prion protein gene PRNP 2.Familial -> less than 10% of CJD is inherited in an autosomal dominant manner, mutation of prion protein gene also 3.Acquired -> all types are transmissible

#13

Front (Question)

What occurs to result in the mutation of the prion protein gene (PRNP)

Back (Answer)

Substitution of one amino acid to another at position 129 of PRNP (prion protein gene)

#14

Front (Question)

3 ways CJD can be acquired

Back (Answer)

• Transmission via exposure to brain / spinal tissue from infected person • Canabalism -> Kuru, a prion disease, among Fore people in Papua New Guinea due to funerary canabalism • Routine sterilisation procedures may fail to inactivate infectious prions

#15

Front (Question)

5 types of prion disease

Back (Answer)

• CJD • vCJD • Kuru • Scrapie -> sheep • BSE/mad cow disease -> cows

#16

Front (Question)

worldwide incidence of CJD

Back (Answer)

1 to 2 cases per one million people per year

#17

Front (Question)

Why is the incidence of CJD increasing (5)

Back (Answer)

• Increased surveillance • Aging population • Increasing population • Clinical awareness • Diagnostic methods

#18

Front (Question)

What is vCJD

Back (Answer)

The human equivalent of mad cow disease

#19

Front (Question)

Aetiology of vCJD (3)

Back (Answer)

• Transmission via eating BSE infected beef • Genetic susceptibility • Blood product transmission possible

#20

Front (Question)

When was the BSE epidemic in Ireland

Back (Answer)

1996

#21

Front (Question)

When did the no. of BSE cases in Ireland peak and how many cases were there

Back (Answer)

The cases peaked in 2002 with 333 cases

#22

Front (Question)

PATHOGENESISSSS When does prion disease occur

Back (Answer)

• When normal prion proteins (found on the surface of many cells) become abnormally folded. • These trigger abnormal folding of other prion proteins causing clumps and consequent brain damage.

#23

Front (Question)

PATHOGENESISSSS What is abnormal folding in terms of protein structure

Back (Answer)

Alpha helices to beta sheets

#24

Front (Question)

PATHOGENESISSSS What does PrPc mean and where is it found

Back (Answer)

PrPc = normal prion protein, a normal consitute of brain tissue in all mammals