Protein Folding III

Question 1

What are the five ways that protein folding problems can cause diseases?

Answer 1

Improper degradation
Improper localization
Dominant Negative mutations
Gain of toxic function
Amyloid accumulation

Question 2

Proteins are quality controlled by (3)

Answer 2

proteasomes, autophagy, ERAD(Endoplasmic reticulum associated degradation)

Question 3

What is a common amyloidgenic protein sequence?

Answer 3

VQIVY

Question 4

Keystones for environmental stressors (3)

Answer 4

DRA: Detect, respond and adopt

Question 5

Improper degradation

Answer 5

When ERAD or Proteasomes don’t do their job correctly since they are “overactive”

CFTR mutation

Question 6

Improper localization

Answer 6

Improper localization due to misfolding

mutation in AAT

Question 7

Dominant Negative Mutation

Answer 7

A mutant protein antagonizes the function of the wild-type protein

monomer mutation has downstream impact as it grows

Question 8

Gain of toxic function

Answer 8

Protein conformation change causes dominant phenotype

EX: Gain of toxicity

Question 9

Amyloid Accumulation

Answer 9

Insolute protein aggregates

Question 10

3 steps of amyloid plaque formation

Answer 10

Seeding or nucleation, fibril formation and finally deposit

Question 11

What is the sequence for amyloidogenic proteins?

Answer 11

VQIVY