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The flashcards below were created by user
victimsofadown
on FreezingBlue Flashcards.
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What stages in RBC development is hemoglobin made in what percent?
- 65% in metarubricyte stage
- 35% in reticulocyte stage
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How many molecules of hgb per RBC? % weight of RBC? % volume of RBC? What is the charge on RBC surface?
- 640 million hgb/RBC
- 95% weight
- 33% volume
- Negative charge due to negative charge of hgb
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What is the structure of a normal hgb molecule? and what is the % of each?
- 4 heme + 4 globin chains
- Protein: 96% (globin chains)
- Heme: 4% (protoporphyrin ring, iron)
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Describe iron delivery to the cell during hemoglobin synthesis in detail
- Transferrin transports iron (Fe3+) into the cell
- Most is transferred to the mitochondria and converted to Fe2+Any excess aggregates in the cytoplasm as ferritin
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Name the stages of hemoglobin synthesis
- Iron delivery to the cell
- Protoporphyrin synthesis
- Globin synthesis
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Describe protoporphyrin synthesis during hemoglobin synthesis in detail
- Succinyl-CoA and glycine enter the mitochondria
- ΔALA (delta aminolevulinic acid) creates porphobilinogen, which is then converted into uroporphyrinogen, then coproporphyrinogen, and finally protoporphyrin
- **ΔALA is the rate limiting step
- Protoporphyrin is combine with Fe and heme is released from the mitochondria
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Which globin chain alleles are on which chromosome?
- Chromosome 11: ε, Gγ (glycine), Aγ (alanine), δ, β
- Chromosome 16: ζ, α, α
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Describe globin chain production amounts from fetus to adulthood
- ζ and ε: embryonic; 100% at 0mo very low at 2mo, ends at 4mo
- γ: fetal; very high at 2 mo, sharply drops 9mo (birth), persists slightly throughout life
- α: very high at 2 mo and persists throughout life
- β: adult; slowly raises to 20% through 9mo (birth) then quickly spikes around 3mo after birth and persists throughout life
- δ: very low development that begins around 8mo and persists (low) throughout life
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What are the various types of abnormal hemoglobins w/ combo of globin chains (detailed)
- H (β4): occurs in alpha thalassemia
- Bart's (γ4): occurs in alpha thalassemia
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What are the various types of normal hemoglobins w/ combo of globin chains (detailed)
- Embryonic hemoglobin
- 3 weeks to 3 months in fetus
- Gowers type 1 (ζ2ε2)
- Gowers type 2 (α2ε2)
- Fetal hemoglobin
- 3 mos in fetus to 9-12mo after birth
- F (α2Aγ2)
- F (α2Gγ2)
- Adult hemoglobin
- Increases after 12mo to normal levels
- A (α2β2)
- A2 (α2δ2)
- A1C (α2[β-NH-glucose]2)
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What is the approximate Hgb composition in a normal adult blood cell?
- Hgb A: 95-97% (includes less than 5% Hgb A1C)
- Hgb A2: 2-3%
- Hgb F: 1-2%
- *note - Hgb F represents 70-90% of all hgb in RBC at birth
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What are the two normal states of hemoglobin and what regulates them?
- Oxyhemoglobin
- Deoxyhemoglobin
- 2,3-diphosophoglycerate (2,3-DPG) regulates O2 affinity
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What are the abnormal hemoglobin states of clinical importance with a brief description
- Carboxyhemoglobin: CO replaces CO2
- *note - CO binds 200x more firmly than O2
- Methemoglobin: oxidized hemoglobin
- (Fe3+ instead of Fe2+)
- Sulfhemoglobin: sulfur replaces iron
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