10 Hemoglobin Metabolism 12.

What is the term best describing an

oxidized hemoglobin?
Methemoglobin
1. Hemoglobin is one of the most studied
protein in the body for what reason?
Globin Structure
Ability to easily isolate it from RBC

2. What percent does hemoglobin occupies in

RBC cytoplasm?
95%
3. What is the role of RBC in maintaining
hemoglobin integrity?
It provides protection from denaturation in
the plasma
Loss through the kidney
13. Globin chain is divided into how many
helices?
4. What is the concentration of hemoglobin
8
within the RBC?
34 g/dL Complete Hemoglobin Molecule
5. What is the MW of hemoglobin?
14. What hemoglobin structure that refers to
64,000 Daltons
the amino acid sequence of the polypeptide
6. Enumerate the two functions of
chains?
hemoglobin?
Primary structure
a. Transports oxygen from the lungs to the
15. What type oh Hgb structure referring to
tissues; transport carbon dioxide from
chain arrangements in helices and
tissues to the lungs
nonhelices?
b. Regulate acid-base balance by binding
Secondary structure
and releasing hydrogen ions and
16. A Hgb structure that refers to the
transport nitric oxide (NO)
arrangement of the helices into a pretzel-
7. What compound that serves as a regulator
like configuration?
of vascular tone?
Tertiary structure
Nitric oxide (NO)
17. Where does heme group in polypeptide
Hemoglobin Structure chain located? What helices?
Between the E and F helices
8. Hemoglobin is the first protein whose
18. What is the significance of having
structure was described using what
hydrophilic amino acids on outside of the
method?
Hgb?
X-ray crystallography
It helps the iron remain in its divalent form
9. Hemoglobin molecule is composed of what?
regardless of whether it is oxygenated or
a. 2- diff pairs of polypeptide chains
deoxygenated
b. 4- heme groups
19. What Hgb structure that describes the
10. What do you call a heme consists of carbon
complete hemoglobin molecule?
ring, hydrogen and nitrogen atoms?
Quaternary structure/ Tetramer
Protoporphyrin IX (9)
20. What is the predominant Hgb molecule in
11. Heme molecule is reversibly combines with
adults?
how many oxygen molecules?
Hemoglobin A
One (1)
21. What are the two alpha-beta bond that
holds the dimers in a stable form?
a1–b1 and a2–b2 bonds
22. What bonds are important for the stability
of the quaternary structure in the
oxygenated
and deoxygenated form?
a1–b2 and a2–b1
23. What term best describes posttranslational
modification formed by the non-enzymatic
binding of various sugars to globin chain
amino groups over the life span of the RBC?
Glycation
24. What is most characterized of the glycated
hemoglobin?
Hbg A1c
25. What percent does Hgb A circulates in the
Hgb A1c form?
4%-6%
26. In uncontrolled diabetes mellitus, what will
happen to the amount of Hbg A1c?
Increased proportionally to the mean blood
glucose level over the preceding 2 to 3
months
27. Where does hemoglobin biosynthesis
occur?
Mitochondria and Cytoplasm of bone
marrow erythrocyte precursor
28. How does heme synthesis begin in the
mitochondria?
condensation of glycine and succinyl
coenzyme A (CoA) catalyzed by
aminolevulinate synthase to form
aminolevulinic acid (ALA)
29. What enzyme that converts ALA to
porphobilinogen (PBG)?
Aminolevulinic acid dehydrogenase
30. What plasma carrier protein that carries
iron in the ferric (Fe3+) form to developing
erythroid cells?
Transferrin
31. What causes the release of iron from
transferrin?
Acidification of endosome
32. What globin genes that are in the short
hand of chromosome 16? a- and z globin
genes
33. What globin gene clusters are in the short
arm of chromosome 11?
e-, g-, d-, and b-globin gene cluster
34. It is composed of two a and b globin chains
and present in 6 months of age through
adulthood?
Hb A
35. Hb A2 comprises _____% of total hemoglobin
in adult
3.5%
36. Hb F comprises_____% of total hemoglobin,
and it is present only in a small proportion
of the RBCs
1% to 2%
37. RBCs with Hb F are called?
F or A/F cells
38. What is used for fractionation, presumptive
identification, and quantification of normal
hemoglobin and hemoglobin variants?
HPLC- high performance liquid
chromatography
39. It provides definitive identification of
variant hemoglobin?
Molecular genetic testing of globin gene
DNA
40. What globin chains normally appear only
during the first 3 months of embryonic
development
z- and e-globin
41. During the second and third trimesters of
fetal life and at birth, what is the
predominant hemoglobin? Hb F (a2g2)
42. What does heme negative feedback means?
a. Heme inhibits the transcription of
the ALA synthase gene, which leads
to a decrease in heme production
b. Heme inhibits other enzymes in the
biosynthesis pathway, including
ALA dehydrase and PBG deaminase
43. A negative feedback mechanism by heme or
substrate inhibition by protoporphyrin IX is
believed to inhibit what enzymes?
ferrochelatase enzyme
44. In globin synthesis, what causes
accumulation and damage the cells,
reducing its lifespan?
Excess of globin chains, protophophyrin IX,
or iron
45. Globin production is mainly controlled at
the transcription level by a complex
interaction of?
a. soluble transcription factors
b. DNA
 46. Initiation of transcription of a particular
globin gene requires?
(1) the promoter DNA sequences immediately
before the 5’ end or the beginning of the gene;
(2) a key transcription factor called Krüppel-like
factor 1 (KLF1);
(3) a number of other transcription factors
(such as GATA1, Ikaros, TAL1, p45-NF-E2, and
LDB1);
(4) an enhancer region of DNAse 1 hypersensitive
nucleic acid sequences located more than 20
kilobases
upstream (before the 5’ start site of the gene) from
the globin
gene called the locus control region or LCR

47. What is the factor that plays a key role in

connecting the complex of LCR, B-globin
gene promoter, and transcription factor.
also plays a key regulatory role in the
switch from g chain to b chain production
(g-b switching) that begins in late fetal life
and continues through adulthood?
Krüppel-like factor 1
48. Reference intervals for hemoglobin
concentration are as follows:
Men: 14 to 18 g/dL (140 to 180 g/L)
Women: 12 to 15 g/dL (120 to 150 g/L)
Newborns: 16.5 to 21.5 g/dL (165 to 215
g/L)
49. Individuals living at high altitudes have
slightly higher levels of hemoglobin as a
compensatory mechanism to provide more
oxygen to the tissues in the oxygen-thin air.
50.