Hemoglobin
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Hemoglobin Overview
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- 65% made in developing RBC through metarubricyte stae (before cells goes to bone marrow, no nucleus)
- 35% made in reticulocyte state (change RNA, making protein)
- 640 million Hgb molecules/RBC
- 95% RBC weight
- 33% RBC v
- Structure of normal hemoglobin
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- 4 heme + 4 globin chains per Hgb molecule
- Each Hgb molecule contains:
- protein 96%
- Globin (polypeptide chains)
- alpha, beta, gamma, delta
- Heme 4%
- protoporphyrin ring and iron<
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Hemoglobin synthesis:
Iron delivery to the cell
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- Iron delievered to Macrophage or RBC
- Transferrin is the carrier protein
- Ferritin: storage product of iron
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Hemoglobin synthesis:
Protoporphyrin synthesis
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- Succiny-CoA + Glycine --> delta-ALA (in mitochondria) --> porphobilinogen --> uroporphyrinogen (URO) --> Coproporphyrinogen
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Hemoglobin synthesis:
Globin synthesis
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- Chromosome 11
- epsilon (embryonic chain)
- G (glycine) gamma
- A (Alanine) gamma
- delta
- Beta
- Chromosome 16
- zeta (embryonic chain)
- 2 alpha chains
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Globin Chain Production
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- 2 months --> Birth: gamma fetal high
- alpha: remain forever in adults in normal Hgb
- beta: high normally produced
- epsilon & zeta: normally appear only during embryonic development
- alpha & delta are constitu
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Normal Hemoglobin:
Embryonic
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- Gower's type 1
- zeta 2 and espilon 2 chains
- Gower's type 2
- alpha 2 and epsilon 2 chains
- 3 weeks to 3 months in fetus
- Gower's type 1
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Normal Hemoglobin:
Fetal
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- F (2 alpha alanine 2 gamma)
- F (2 alpha glycine 2 gamma)
- 3 months in fetus to 9-12 months after birth
- doesn't give off oxygen as it should if adults has Hgb F
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Normal Hemoglobin:
Adult
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- A (alpha 2 beta 2) 95-97% (includes less than 5% Hgb A1C)
- A2 (alpha 2 delta 2) 2-3%
- A1C
- alpha 2 ( B-NH-Glucose)2
- diabetes indication
- if more than 10%
- increases after 12 months
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Other hemoglobin
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- H (4 Beta chains)
- occurs in alpha thalassemia
- don't make A chains
- Bart's (4 gamma chains)0
- occurs in alpha thalassemia
- Functions of Hemoglobin
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- oxyhemoglobin:
- an important control of O2 affinity is 2,3-diphosphoglyerate (2,3-DPG)
- not oxidized hemoglobin
- deoxyhemoglobin
- hemoglobin without oxygen
- carboxyhemoglobin:
- oxyhemoglobin: