Flashcards in hemoglobin Deck (13):
mean hemoglobin per cell in picograms
most hemoglobin synthesis occurs during this stage:
15 g /dL blood
condensation of glycine and succinyl CoA. occurs only in presence cofactor pyridoxal phosphate and the enzyme 5-aminolevulinate synthase (ALAS). Rate limiting step only occurs in presence of iron. This step occurs in mictochondria, exits to cyoplasm to form Coproporphyrinogen then reenters mitochondria to form protoporyhyrin IX.
chelates iron with protoporyphyrin Ix
fetal alpha equivalant. very early embryonic development.
short arm chromosome 16
zeta and alpha location. two alpha loci.
Beta like genes
located in order of activation on chromosome 11. E, Gamma, D and B.
non alpha affinity for alpha
Beta > gamma > delta
AHSP alpha hemoglobin stabilizing protein
AHSP binds free a@chains and stabilizes their structure, increasing their affinity for b@chains. This accelerates the formation of hemoglobin tetramers.
McKenzie, Shirlyn B.; Williams, Lynne. Clinical Laboratory Hematology (3rd Edition) (Pearson Clinical Laboratory Science Series) (Page 82). Prentice Hall. Kindle Edition.
Heme binding position
6 electrons in iron. four bind heme, 1 binds proimal histidine F8 other is for oxygen. Located between proximal and distal E8 histidine.
Regulation of hemoglobin synthesis
activity concnetration of ALAS2,
Activity of porphobilinogen deaminase (PBGD) • Concentration of iron • Regulation of globin chain synthesis