Amyloid Diseases

Question 1

Amyloid diseases

Answer 1

Involve deposition of proteins within tissue

Naturally occuring protein (usually globular) unfolds, aggregates and forms fibrous plaques

Question 2

Amyloid plaques

Answer 2

Formed from beta-sheet proteins

Question 3

Systemic Amyloid Diseases

Answer 3

Excessive amount of antibody light chains accumulate as amyloid deposits in different tissues
- commonly kidneys
-e.g antibody light chain amyloidosis (AL amyloidsosis)

Build up in heart can cause heart failure
Tend to unfold in bloodstream when bloodflow is fastest

Question 4

Acute Phase Proteins

Answer 4

Proteins whose serum concentration increase in response to inflammation

i.e. serum amyloid A

Question 5

Serum amyloid A

Answer 5

recruits immune cells and transports cholesterol to the liver
Upregulated in chronic inflammation so builds up

The more protein, the more likely it is to be unfolded, more likely to aggregate, forms amyloid deposits

Question 6

Amyloid Diseases: Autoimmune diseases

Answer 6

Involve chronic inflammation due to immune response mistankenly attacking healthy tissue

i.e. rheumatoid arthritis, Crohn’s disease

Question 7

Chronic inflammatory disease

Answer 7

Often present alongside amyloid deposits due to high concentration of serum-amyloid A in area

Question 8

Dialysis-related amyloidogenesis

Answer 8

Iatrogenic disease (disease caused by medical treatment)

In long-term dialysis proteins build up and unfolded when inside dialysis tubes - specifically beta2-microglobulin (component of MHCI) deposits in organs and joints

Question 9

Iatrogenic disease

Answer 9

induced by or resulting from a medical treatment

Question 10

Dialysis-related amyloidogenesis (DrA) solution

Answer 10

No longer a problem
Developments in dialysis improved the clearance of beta2-microglobulin

Question 11

Type 2 Diabetes

as an amyloid-related disease

Answer 11

Consequence of insulin resistance
Body tries to increase expression of insulin to combat this

Hormone called islet amyloid polypeptide (IAPP) is expressed at the same time as insulin but x100 more

Question 12

IAPP

Answer 12

islet amyloid polypeptide
- very small protein so is not a folded , it is involved in glucose homeostasis

high [IAPP] –> deposits in islet cells

Question 13

General idea of amyloid formation

Answer 13

More protein measn that there is a higher likelihood for the protein to be unfolded

More unfolded proteins
More aggregation

Amyloid formation