Myoglobin and Hemoglobin Flashcards
How long will you see embryonic Hb present?
~ 1 week post-conception until birth
CO + heme in Hb (HbCO)=
Carboxyhemoglobin –>forms more readily than oxyhemoglobin (HbO2)
What is the most common Hb variant associated with pathology in the US? What disease does it cause?
Bs mutation; sickle cell anemia
Methemoglobinemia is characterized by
Chocolate blood and cyanosis
What are 4 substances that competitively bind to the Fe-binding site in heme and which one binds up to 300x stronger than O2?
- Cyanide (CN-)
- Carbon monoxide (CO)
- Nitrogen dioxide (NO2)
- Hydrogen sulfide (H2S)
CO binds very strongly to Hb
Mutations in cytochrome b5 reductase cause hereditary ________. What is the purpose of this enzyme?
This enzyme uses NADH generated through glyceraldehyde phosphate dehydrogenase (glycolysis) to reduce Fe3+ in methemoglobin to Fe2+ thereby reversing formation of Fe3+.
alpha2 delta2
HbA2
2,3-DPG (same as 1,3-BPG) is present in all RBCs in adults and is produced in RBCs when _____ is abundant and stabilizes the __ state
glucose; T
Most CO2 enters RBCs and 15 % of the time forms _____ _____with Hb or is converted to H2CO2 (85% of the time) by _____ ______ then dissociates to HCO3- and H+.
carbamate adducts; carbonic anhydrase
alpha2 gamma2
HbF
p50 of Hb
26 torr
At position 6 of the B-globin gene, the amino acid change from ___ to Val6 confers sickle cell anemia
Glu6
Sigmoidal binding curve = what type of cooperativity?
Positive; in Hb
Alveolar partial pressure of O2 is ___ while the alveolar partial pressure of CO2 is ___
100 mmHg; 40 mmHg
In the lungs, high pO2 drives binding of O2 to Hb, release of H+, and formation of H2CO3 (carbonic acid). Carbonic acid equilibrium favors synthesis of CO2 and H2O. What is the name of this effect?
Haldane Effect
p50 of Mb
2.8 torr
Fe-protoporphyrin IX =
Heme
Weak ____ interactions are between the a1B1 and a2B2 heterodimers in Hb
Polar
Myoglobin is made up of primarily what conformation of protein?
Alpha helices - 80%
Hb Bart’s disease or hydrops fetalis is formed by how many alpha globin deletions?
4
O2 binds to Hb _____
Cooperatively