Amyloid disease

Question 1

Chronic degenerative diseases are associated with…

Answer 1

Accumulation of abnormally folded proteins

Question 2

What are associated pathological changes with abnormally folded proteins?

Answer 2

Specific protein affected, target tissue, cell loss, loss of function

Chronic inflammation as mechanism of tissue damage

Question 3

What does amyloid stain with?

Answer 3

Congo red

Question 4

What is senile systemic disease associated with?

Answer 4

Build up to transthyretin, CHF

Question 5

What is dialysis associated protein?

Answer 5

B2 microglobulin is damaged by haemodialysis

Question 6

What is the pathology of dialysis associated degeneration?

Answer 6

Carpal tunnel syndrome, deposits in bone and tendons, joint effusion and arthritic pain

Question 7

What proteins are associated with familial visceral diseases? What is the cause?

Answer 7

Fibrinogen, alpha chain, apoL1, lysozyme

Genetic (misfolding risk)

Question 8

What protein is associated with type II diabetes?

Answer 8

37-mer Islet amyloid precursor polypeptide (co-secreted with insulin)

Question 9

What proteins are associated with Alzheimer’s ?

Answer 9

Amyloid B APP

Presenilin 1/2 genes

Question 10

What protein is associated with Parkinson’s ?

Answer 10

A-synuclein

Question 11

What proteins are associated with amyloidoma ?

Answer 11

Ig light chains

Question 12

What proteins are associated with Creutzveldt-Jakob disease?

Answer 12

Prions (spontaneous)

Question 13

What proteins are associated with vCJD?

Answer 13

Prions (infectious)

Question 14

What proteins are associated with familial fatal insomnia?

Answer 14

Prions (genetic)

Question 15

What are prion diseases?

Answer 15

Chronic neurodegenerative diseases characterised by extensive neuronal loss without replacement by glial cells, or ECM

Question 16

How do prion diseases look in histology?

Answer 16

Leave large acellular areas clearly seen as vacuolated in histological sections.

Question 17

Symptoms of creutzveldt-jakob disease?

Answer 17

Rapidly developing delirium or dementia (over the course of a few weeks or months), Blurred vision (sometimes), Changes in gait, Hallucinations, Lack of coordination (for example, stumbling and falling), Muscle twitching, Muscle stiffness, Myoclonic jerks or seizures, Nervous, jumpy feelings, Personality changes, Profound confusion, disorientation, Sleepiness and Speech impairment

Question 18

What is a prion?

Answer 18

A small proteinaceous infectious particle which resists inactivation by procedures that modify nucleic acids