Haemoglobin Structure And Function Flashcards
(43 cards)
What is haemoglobin made up of?
2 alpha and two beta chains
How much of RBCs is comprised of a globular haemoprotein
1/3
What is a haemoprotein?
A group of specialised proteins that contain haem as a tightly bound prosthetic group
What is haem a complex of?
Protoporphyrin IX and ferrous iron Fe2+
How is the iron held in the centre of the haem molecule?
Bonds to the 4 nitrogens of a porphyrin ring
How much Hb is synthesised in the erythroblasts?
65%
How much Hb is synthesised at the reticulocyte stage?
35%
What is haemoglobin synthesis stimulated by?
Tissue hypoxia (lack of oxygen)
How is Hb synthesis stimulated by tissue hypoxia?
Hypoxia causes the kidneys to increase EPO production which increases RBC and Hb production
What does EPO Stand for?
Erythropoietin
What molecule delivers iron to the reticulocyte?
Transferrin
Where does synthesis of protoporphyrins take place?
In the mitochondria of RBC precursors
What is synthesis of protoporphyrins mediated by?
EPO and vitamin B6
Protoporphyrin + iron->
Haem
What are the starting reactants in the synthesis of protoporphyrin?
Glycine + succinyl CoA
What is the iron stored as if you don’t need it?
Ferritin
Where does synthesis of globin happen?
In the ribosome and RER
What does proper globin synthesis depend on?
Genes
How many functional globin chains are there?
8
What are the two clusters of globin called?
A and B
What genes are in the B-cluster?
Beta, gamma, epsilon and delta
Where are the B-cluster genes found?
On the short arm of chromosome 11
What genes are in the A-cluster?
Alpha and zeta
Where are the A-cluster genes found?
Short arm of chromosome 16