HEMOGLOBIN
Iron bearing protein contained within the _________
It is synthesized by _________ from the________ up to_________
1 gram of hemoglobin can carry_______
Erythrocytes
Young erythroblast ; polychromatophilic normoblast stage ; reticulocytes stage
1.34 mL of oxygen
Functions of hemoglobin:
Transport oxygen from the_____ to the_______ and carbon dioxide from the__________
Base balance regulation
Lungs
Tissue
tissue
Acid
Composition of heme (2)
Protophorphyrin IX
Ferrous iron
Protophorphyrin consists of a ring of: (3)
Carbon
Hydrogen
Nitrogen
Structure of hemoglobin:
a1B2 & a2 B1 bonds:
a1 B1 bonds:
a2 B2 bonds:
Between the dimers
Front
Behind
Globin structure:
_______ compromising each hemoglobin molecule consist of_______ of unlike polypeptide chains____to______
Each hemoglobin_______ separated by_______
Four globin chains
Two identical pairs
141 to 146 amino acids each
8 helices ; 7 non helices segments
Number of amino acids:
Alpha & Zeta:
Beta, Gamma, Delta ,epsilon:
Theta:
141 amino acids
146 amino acids
Unknown
Complete hemoglobin molecule
Primary:
Secondary:
Tertiary:
Quaternary:
Amino acid sequence
Helices and non-helices
Pretzel like configuration
Complete molecule
Outline of heme synthesis:
Succinyl- CoA + Glycine —-> ALA synthase = ________
______ enters cytoplasm by ________then becomes______then_______then_____——->_________ then becomes______ enters mitochondria again by ________ then becomes______ then______ then_______ to form _______
Amino levulinic acid
Amino levulinic acid ; ALA-dehydratase ; porphobilinogen ; hydroxymethylbilane ; uroporphorinogen III ; uroporphirinogen III decarboxylase ; coproporphyrinogen III ; coproporphyrinogen III oxidase ; Protoporphyrinogen III ; protoporphyrin IX ; ferrochelatase ; HEME
Ontogeny of globin synthesis:
3 weeks of gestation
-region:
-type of globin gene:
-type of hemoglobin:
5 weeks of gestation
-region:
-type of globin gene:
-type of hemoglobin:
6 - 30 weeks of gestation
-region:
-type of globin gene:
-type of hemoglobin:
30 weeks of gestation
-region:
-type of globin gene:
-type of hemoglobin:
At birth
-region:
-type of globin gene:
-type of hemoglobin:
Yolk sac
Zeta and epsilon
Hemoglobin gower 1
Yolk sac
Gamma and Alpha
Hemoglobin Portland and hemoglobin gower 2
Liver and spleen
Alpha, Gamma, Beta
Hemoglobin F
Liver
Delta
Hemoglobin A2
Bone marrow
None
Hemoglobin A
Function of hemoglobin is readily bind oxygen molecules in lung, which requires high oxygen affinity to transport oxygen into efficiently unload oxygen to the tissues which requires low oxygen affinity
Oxygen transport
Second crucial function of hemoglobin is the transport of carbon dioxide
Carbon dioxide
Third function of hemoglobin involves the binding and activation and transport of nitric oxide
Secreted by vascular endothelial cells and causes relaxation of vascular walls, smooth muscle and muscle vasodilation
Nitric oxide
Ability of hemoglobin to bind or release oxygen. Oxygen tension at hgb is 50% saturated
Oxygen affinity
Relationship between O2 tension amd hemoglobin saturation with oxygen
Oxygen Dissociation Curve
Relationship of O2 affinity with hgb to pH
Bohr effect
Increase pH:
Decrease pH:
Increase hgb affinity for O2
Decrease affinity for O2
Shift to the left (DECREASE) (3)
TEMPERATURE
ORGANIC PHOSPHATES (2,3 DPG)
p (CO2)
Shift to the left (INCREASE) (2)
p (CO)
pH
Shift to the right (DECREASE)
p (CO2)
pH
Shift to the right (INCREASE) (3)
TEMPERATURE
ORG PHOSPHATE (2,3 DPG)
p (CO2)
________ ( decreases oxygen affinity, more O2 release)
(4)
________( increases oxygen affinity, less O2 release)
(3)
Right shift
High 2,3 DPG
Decrease body pH
High carbon dioxide
Hemoglobin S
Left shift
Low 2,3 DPG
Decrease body temp
Hemoglobin F
Hemoglobin combination with oxygen
Give pinkness to skin and mucous membrane.
Seen in arterial circulation
Oxyhemoglobin
Hemoglobin with iron but no O2 seen in venous circulation
Unassociated with oxygen
Deoxyhemoglobin