Hemoglobin structure

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20 Terms

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zeta and epsilon

chains that peak shortly after conception, decline dramatically and are not detectable after 3 months in utero

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alpha chains

begin at approximately 19 days post-conception, rise rapidly in the first 3 months of gestation, and remain throughout life

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gamma chains

begin shortly after conception, rise rapidly to 4-5 month gestation, decrease at birth adn are berely detectable after 6 months

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beta chains

begin shortly after conception, rise slowly during gestation, reach significant levelt at 3 months, peak ata 6 months and remain throughout life

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delta chains

begin about 6 months in utero, plateau at 6 months and remain at this level throughout life

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Hgb A

alpha and beta chains
97% total hbg in adults
20% total hbg in newborns

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Hgb A2

alpha and delta chains
2.5% total hbg in adults
<1% total hbg in newborns

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Hgb F

alpha and gamma chains
<1% total hbg in adults
80% total hbg in newborns

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Hgb Gower 1

zeta and epsilon chains
embryonic hgb

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Hgb gower 2

alpha and epsilon chains
embryonic hgb

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Hgb Portland

zeta and gamma chains
embryonic hgb

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Thalassemia

disease in which one or more of the 6 normal globin genes are mutated resulting in a deficiency of one or more globin chains reducing the corresponding type of hemoglobin

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Alpha thalassemia

affect HbA, HbA2, and HbF

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Beta thalassemia

Affects HbA

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secondary structure

alpha helix vs beta pleated sheet, proline kinks

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tertiary structure

3D folding pattern
Globular

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Quaternary structure

tetrameric
2 alpha-like chains
2 beta like chains

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contact points

alpha1beta1 to alpha2beta2

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beta gap

pocket in the inner core of the globin chains
enlarges in deoxyhemoglobin

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allosteric change

movement of the globin molecules as hemoglobin converts from oxyhemoglobin to deoxyhemoglobin