Amyloid disease Flashcards
Chronic degenerative diseases are associated with…
Accumulation of abnormally folded proteins
What are associated pathological changes with abnormally folded proteins?
Specific protein affected, target tissue, cell loss, loss of function
Chronic inflammation as mechanism of tissue damage
What does amyloid stain with?
Congo red
What is senile systemic disease associated with?
Build up to transthyretin, CHF
What is dialysis associated protein?
B2 microglobulin is damaged by haemodialysis
What is the pathology of dialysis associated degeneration?
Carpal tunnel syndrome, deposits in bone and tendons, joint effusion and arthritic pain
What proteins are associated with familial visceral diseases? What is the cause?
Fibrinogen, alpha chain, apoL1, lysozyme
Genetic (misfolding risk)
What protein is associated with type II diabetes?
37-mer Islet amyloid precursor polypeptide (co-secreted with insulin)
What proteins are associated with Alzheimer’s ?
Amyloid B APP
Presenilin 1/2 genes
What protein is associated with Parkinson’s ?
A-synuclein
What proteins are associated with amyloidoma ?
Ig light chains
What proteins are associated with Creutzveldt-Jakob disease?
Prions (spontaneous)
What proteins are associated with vCJD?
Prions (infectious)
What proteins are associated with familial fatal insomnia?
Prions (genetic)
What are prion diseases?
Chronic neurodegenerative diseases characterised by extensive neuronal loss without replacement by glial cells, or ECM
How do prion diseases look in histology?
Leave large acellular areas clearly seen as vacuolated in histological sections.
Symptoms of creutzveldt-jakob disease?
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
What is a prion?
A small proteinaceous infectious particle which resists inactivation by procedures that modify nucleic acids
