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Hematology I > Hemoglobin synthesis > Flashcards

Hemoglobin synthesis Flashcards

1
Q
  • main component of RBC (95%)
  • respiratory pigment
  • 4 heme, 4 polypeptide chains
A

Hemoglobin

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2
Q

main function of hemoglobin

A

responsible for oxygen & carbon dioxide carrying capacity of RBC

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3
Q

primary function of rbc

A

manufacture Hgb

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4
Q
  • enzymatic reaction

- begins in mitochondria

A

heme synthesis

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5
Q
  • genetic control

- ribosomes in the cytoplasm

A

globin synthesis

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6
Q

1 RBC= how many Hgb

A

34 g/dL Hb

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7
Q

1 gm of Hgb can carry how many O2

A

1.34 mL

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8
Q

1 gm of Hgb carries a constant ____ iron

A

3.47 mg

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9
Q

Hgb molecule is commposed of four subunits, each containing ___ and ____

A

heme, globin

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10
Q
  • belongs to the class of pigment

- is a porphyrin ring w/ an iron atom at its center

A

heme

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11
Q

heme is also known as

A

protoporphyrin IX or ferroprotoporphyrin IX

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12
Q

most functional protein in iron

A

hemoglobin

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13
Q

most storage protein in iron

A

ferritin

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14
Q

most transport protein in iron

A

transferrin

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15
Q

site where maximum iron absoprtion

A

duodenum & jejunum

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16
Q

membrane protein in enterocyte tansports iron from intestinal lumen into the enterocyte

A

divalent metal transporter 1

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17
Q

membrane protein that transports iron from macrophage, hepatocytes & enterocytes going towards the blood

A

Ferroportin

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18
Q

responsible for the transport of iron in plasma

A

apotransferrin/transferrin

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19
Q

saves free hem & hemoglobin especially in hemolysis

A

haptoglobin

hemopexin

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20
Q

absorption of ionic iron in the _____

A

small intestine

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21
Q

ferric iron reduction by ____

A

DMT1

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22
Q

ferrous iron oxidation by ______

A

hephaestin

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23
Q

stronger form of iron

A

ferric

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24
Q

functional form of iron

A

ferrous

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25
Q

DMT1 transfer iron into ____

A

cytosol

26
Q

iron is stored as ____

A

ferritin

27
Q

inactive the absorption & recycling of iron by acting on enterocytes, macrophages & hepatocytes

A

hepcidin

28
Q

most functional iron in humans

A

hemoglobin

myoglobin

29
Q

how many iron is in storage form

A

1/4

30
Q

average american diet iron/day

A

10-20 mg

31
Q

average american diet iron/day that is absorbed

A

1-2 mg

32
Q

iron is absorbed from the ___

A

gut

33
Q

comprises of 141-146 amino acids

A

globin

34
Q

heme synthesis occurs in the ___

A

bone marrow

35
Q

heme synthesis is from ____ stage to ____ stage

A

pronormoblast, reticulocyte

36
Q

synthesis is under genetic control

A

globin synthesis

37
Q

alpha and zeta genes of globin is from ___

A

chromosome 16

38
Q

beta, delta, epsilon & gamma is from ___

A

chromosome 11

39
Q

the production of globin chains takes place in

A

pronormoblast-polychromatophilic erythrocyte or immature RBC

40
Q

embryonic normal hemoglobins

A
  • gower I
  • portland
  • gower II
41
Q

fetal normal hemoglobin

A

HbF

42
Q

variants of normal hemoglobin (adult)

A
  • HbA1

- HbA2

43
Q
  • 95% of adult normal hemoglobin

- post natal life

A

HbA1

44
Q
  • less than 3.5% of adult normal hemoglobin

- small amnt only is found in RBCs

A

HbA2

45
Q

formed by post synthetic, non-enzymatic reactions of various sugars w/ amino groups of the globin chains

A

HbA1c

46
Q

normal & functional hemoglobin

A
  1. Oxyhemoglobin (HbO2)

2. Deoxygenated or Reduced Hemoglobin (HbCO2)

47
Q
  • in arterial blood

- bright red in color

A

Oxyhemoglobin

48
Q
  • in venous blood

- dark red in color

A

Reduced hemoglobin

49
Q

unable to act as an oxygen carrier

A

abnormal or non functional hemoglobin

50
Q

abnormal or non functional hemoglobins

A
  1. carboxyhemoglobin (hbco)
  2. methemoglobin
  3. sulfhemoglobin
51
Q
  • hemoglobin bound to carbon monoxide
  • REVERSIBLE
  • affinity of Hb for CO is 240x greater than for oxygen at 37C
  • hypoxia because HbCO is not capable of transporting oxygen
  • elevated in heavy smokers
A

Carboxyhemoglobin

52
Q

other name of methemoglobin

A

ferrihemoglobin

53
Q
  • contains iron in the ferric state
  • REVERSIBLE because it is unstable
  • when Hb is exposed to oxidizing agent
  • if inc more than 30% px is with hypoxia and cyanosis
A

Methemoglobin

54
Q

methemoglobin increases in:

A
  1. presence of oxidants (nitrite)
  2. dec activity of methemoglobin reductase (gene defect)
  3. mutations in the gene fo r NADH-cytochrome b5 reductase 3 (CYB5R3)
  4. Hb M disease
55
Q

treatment of methemoglobin

A

a. removal of offending subs

b. administration of ascorbic acid or methylene blue (IV)

56
Q

color of blood with carboxyhemoglobin

A

cherry red

57
Q

color of blood with methemoglobin

A

chocolate brown

58
Q

color of blood in vitro with sulfhemoglobin

A

green pigment

59
Q
  • caused by chemical or drugs like aromatic amines or sulfonamides
  • IRREVERSIBLE
  • caused by prolonged constipation, sulfa drugs or trinitrotoluene
A

Sulfhemoglobin

60
Q

RBCs containing HbS have _____ & _____

A

normal survival & osmotic fragility

Hematology I (11 decks)

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