peptides and protein structure-clinical correlates-Brar

Question 1

sickle cell

Answer 1

In sickle cell, Glu 6 in the beta
chain is mutated to a valine–> creates a hydrophobic patch on the surface of hemoglobin–> promotes polymerization–> sickling

Question 2

chaperones

Answer 2

help the protein reach it’s final 3-D structure by helping it overcome the kinetic barriers of high energy states it must go through to get there.

primary structure determines the folding pattern

Question 3

prions

Answer 3

Prion stands for proteinaceous infectious agent

Thought to be responsible for
transmissible spongiform
encephalopathies

PcP(Sc) is the main component of
the infectious agent

This part is also a validated marker
for the disease

Normal protein contains almost no beta sheet structure and is a monomer.
Abnormal protein has extensive beta sheet character and its conformation promotes aggregation

infected PrP in contact with non-infected PrP will cause the normal PrP to fold into the infectious form–> exponential growth