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Flashcards in Amyloid disease Deck (18)
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1
Q

Chronic degenerative diseases are associated with…

A

Accumulation of abnormally folded proteins

2
Q

What are associated pathological changes with abnormally folded proteins?

A

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

Chronic inflammation as mechanism of tissue damage

3
Q

What does amyloid stain with?

A

Congo red

4
Q

What is senile systemic disease associated with?

A

Build up to transthyretin, CHF

5
Q

What is dialysis associated protein?

A

B2 microglobulin is damaged by haemodialysis

6
Q

What is the pathology of dialysis associated degeneration?

A

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

7
Q

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

A

Fibrinogen, alpha chain, apoL1, lysozyme

Genetic (misfolding risk)

8
Q

What protein is associated with type II diabetes?

A

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

9
Q

What proteins are associated with Alzheimer’s ?

A

Amyloid B APP

Presenilin 1/2 genes

10
Q

What protein is associated with Parkinson’s ?

A

A-synuclein

11
Q

What proteins are associated with amyloidoma ?

A

Ig light chains

12
Q

What proteins are associated with Creutzveldt-Jakob disease?

A

Prions (spontaneous)

13
Q

What proteins are associated with vCJD?

A

Prions (infectious)

14
Q

What proteins are associated with familial fatal insomnia?

A

Prions (genetic)

15
Q

What are prion diseases?

A

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

16
Q

How do prion diseases look in histology?

A

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

17
Q

Symptoms of creutzveldt-jakob disease?

A

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

18
Q

What is a prion?

A

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