HEMOGLOBIN Flashcards

1
Q

hemoglobin is a tetrameter composed of how many diff pair of globin polypeptide chain?

A

2 pairs

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2
Q

Hgb has 2 globin chains that binds a heme group containing ?

A

central ferrous iron

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3
Q

Hgb is the 1st protein whose structure was described using x-ray ____

A

crystallography

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4
Q

Hgb is __% of RBCs cystoplasmic content

A

95%

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5
Q

transport molecular oxygen from the lungs to the tissue

A

Hgb

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6
Q

Hgb structure ?

A

4 globin chains and
4 heme group

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7
Q

Hgb MW?

A

64,000 D

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8
Q

Heme biosynthesis occurs in?

A

mitochondria and cytoplasm

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9
Q

Heme biosynthesis starts in ?

A

Mitochondria

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10
Q

Heme biosynthesis. In mitochondria, condensation begins with ?

A

glycine and succinyl coenzyme A

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11
Q

In heme biosynthesis, what is the enzyme between /from condensation to aminolevulinic acid?

A

Aminolevulinic acid synthase

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12
Q

In heme biosynthesis, what are the 3 enzyme from PBG to Coproporphyrinogen III?

A
  1. PBG deaminase
  2. Uroporphobilinogen III synthase
  3. Uroporphobilinogen III decarboxylase
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13
Q

In heme biosynthesis, the enzyme from coproporphyrinogen III to protoporphyrinogen IX?

A

Corprophophyrinogen oxidase

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14
Q

In heme biosynthesis, the enzyme from protoporphyrinogen IX to Protoporphyrin IX?

A

Protoporphyrinogen oxidase

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15
Q

in heme biosyntesis, the enzyme from protoporphyrin IX to HEME

A

ferroc helatase

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16
Q

another name of heme/end product of heme biosynthesis

A

Ferroprotoporphyrin IX

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17
Q

last site in heme biosynthesis

A

cytoplasm

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18
Q

2 zeta, 2 epsilon = ?

A

Gower I

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19
Q

2 alpha, 2 epsilon= ?

A

Gower II

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20
Q

2 zeta, 2 gamma =?

A

portland

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21
Q

2 alpha, 2 gamma = ?

A

Fetal Hgb (HbF)

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22
Q

2 alpha, 2 beta = ?

A

A1

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23
Q

2 alpha, 2 delta =?

A

A2

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24
Q

HbA concentration in adults?

A

92-95%

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25
Q

HbA2 concentration in adults?

A

2-3%

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26
Q

HbF concentration in adults?

A

1-2%

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27
Q

1g of Hb= ___ mL of oxygen

A

1.34 mL

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28
Q

the amount of oxygen needed to saturate 50% of hemoglobin

A

P50

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29
Q

Hemoglobin protein structure. Amino acid sequence of polypeptide chain

A

Primary

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30
Q

Hemoglobin protein structure. Chain arrangements in helices and nonhelices

A

Secondary

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31
Q

Hemoglobin protein structure. Pretzel-like configuration

A

Tertiary

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32
Q

Hemoglobin protein structure. tetrameter, spherical. Described the Hgb structure

A

Quaternary

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33
Q

production of globin chains takes place in ?

A

erythroid precursors

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34
Q

production of globin chains takes place in erythroid precursors from ___ through __

A

Pronormoblast
Polychromatic erythrocyte

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35
Q

3 steps in globin biosynthesis

A

Transcription
Translation
Release

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36
Q

Globin biosynthesis. Transcription occurs in?

A

nucleus

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37
Q

Globin biosynthesis. Globin chains transcription to __

A

mRNA

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38
Q

Globin biosynthesis. translation occurs in ?

A

ribosome

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39
Q

Globin biosynthesis. mRNA translation to ___

A

globin polypeptide chain

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40
Q

Globin biosynthesis. . After translation, the globin chains are released from the ribosome to ___

A

cytoplasm

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41
Q

in genetic coding, what chromosome is the short arm, involvement of Alpha and Zeta

A

Chromosome 16

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42
Q

In genetic coding, what is the long arm, involvement of Beta, Epsilon, Delta and Gamma

A

Chromosome 11

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43
Q

Number of amino acids in ALPHA

A

141

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44
Q

Number of amino acids in BETA

A

146

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45
Q

Number of amino acids in GAMMA (position 136:___)

A

146, alanine

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46
Q

Number of amino acids in DELTA (position 136: ___)

A

146, glycine

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47
Q

Number of amino acids in EPSILON

A

146

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48
Q

Number of amino acids in ZETA

A

141

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49
Q

curve that describe the relationship between oxygen content and partial pressure oxygen

A

Oxygen Dissociating Curve

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50
Q

Oxygen Dissociating Curve. Normal Shape?

A

Sigmoid

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51
Q

defined as PO2 required to produce half saturation of hgb

A

P50 value

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52
Q

normal p50 value, associated with ph ? temp?

A

26.53 mmHg, pH- 7.4, 37.5

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53
Q

increase in o2 affinity therefore less oxygen is released. what shift?

A

shift to left

54
Q

decreased O2 affinity therefore more oxygen is released. what shift?

A

shift to the tight

55
Q

high fever, acidosis, what shift?

A

shift to the right

56
Q

akalosis, methemoglobinemia. what shift?

A

shift to the left

57
Q

increased pH, decreased temp . what shift

A

shift to the left

58
Q

decreased pH and increased temp. what shift?

A

shift to the right

59
Q

this disease is caused by HbS, oxygen supply is decreased

A

Sickle cell disease

60
Q

slighty decreased o2 concentration of HbS- 90-100%

A

sickle cell trait

61
Q

much decreased O2, concnetration of Hbs is 40-45% and HbA is 55-50%

A

Sickle cell anemia

62
Q

quantitative defect in Hgb is called

A

thalassemia

63
Q

genetic disorder which is know for reduction of globin chain synthesis

A

thalassemia

64
Q

Types of qunatitative defect in Hgb (thalassemia)

A
  1. Alpha thalassemia
  2. Beta thalassemia
65
Q

type of thalassemia where there is complete absence of alpha chains and no normal Hb is produced

A

Alpha thalassemia (Major)

66
Q

Alpha thalassemia disease?

A

Hydrops fetalis

67
Q

In Alpha thalassemia (Major), __% of Bart’s Hgb (4 gamma) produced,

A

80%

68
Q

2 alpha chains are deleted in this type of thalassemia

A

Alpha thalassemia (Minor)

69
Q

Type of thalassemia where there is complete absence of beta chains, compensated with up to 90% HbF

A

Beta thalassemia (Major)

70
Q

Major beta thalassemia disease

A

severe microcytic, hypochromic anemia

71
Q

minor beta thalassemia disease

A

mild microcytic, hypochromic anemia

72
Q

one beta chain is normal, compensated with HbA2, what type of thalassemia

A

Beta thalassemia (Minor)

73
Q

one of thee test used to diagnose and follow the treatment of anemia

A

hemoglobin

74
Q

Hgb normal value at birth

A

15-20 g/dL

75
Q

Hgb normal value women

A

12-16 g/dL

76
Q

Hgb normal value men

A

13-18 g/dL

77
Q

Hgb fluctuations. its is ___ in the morning, when exercising and in high altitudes

A

higher

78
Q

Hgb fluctuations. its is ___ in the afternoon, in supine position, dehydration and smokers

A

lower

79
Q

Methods in hemoglobin

A
  1. Colorimetric method
  2. Photoelectric method
  3. Gasometric method
  4. Gravimetric method
80
Q

types of colorimetric methods

A
  1. Acid hematin (Sahli method)
  2. Alkali hematin (Betke test)
81
Q

this colorimetric method uses 0.1HCl reagent for comparing the brownish yellow color of solution

A

Acid Hematin (Sahli Method)

82
Q

This colorimetric method uses 0.1 NaOH, cant be use to alkali resistant therefore cant be used for determination of HbF

A

Alkaki hematin (Betke test)

83
Q

type of photoelectric method which is the reference methos approved by CLSI

A

Cyanmethemoglobin

84
Q

Principle of Cyanmethemoglobin

A

blood is diluted in alkaline drabkin solution of pottasium ferricyanide, pottasium cyanide, sodium bicarbonate and a sufractant

85
Q

original drabkins reagent?

A

sodium hydrogen carbonate

86
Q

original drabkins reagent reaction time?

A

15 mins

87
Q

in Cyanmethemoglobin, only __ is not measured

A

sulfhemoglobin

88
Q

type of Hgb. oxygenated form. scarlet red. arterial blood

A

oxyhemoglobin

89
Q

type of Hgb. deoxygenated form. dark red. venous blood

A

deoxyhemoglobin

90
Q

type of Hgb. combination of hb and CO. reversible. cherry red,

A

carboxyhemoglobin

91
Q

peak absorbance of carboxyhemoglobin

A

576 nm

92
Q

type of Hgb. Oxidized hb. losses its capacity to carry O2 or CO2. iron in ferric state. reversible

A

Methemoglobin

93
Q

color of methemoglobin

A

choco brown

94
Q

methemoglobin peak absorbance

A

630 nm

95
Q

stype of Hgb. stable abnormal Hb pigment. Stable for 9 mons to 6 yrs

A

Cyanmethemoglobin

96
Q

type of Hgb. combination of inorganic sulfides and Hb. irreversible. leads to death of RBC

A

sulfhemoglobin

97
Q

color of sulfhemoglobin

A

mauve lavender

98
Q

sulfhemoglobin peak absorbance

A

618 nm

99
Q

Gasometric method. wherein this method has the greatest accuracy. estimates oxygen-combining power of blood

A

Van Slyke Oxygen Capacity Method

100
Q

Gravimetric method. used method in blood donor screening.

A

Copper sulfate method

101
Q

in Copper sulfate method, if the hb is equal to 12.5g, the drop of blood will sink within ___ and donor is accepted

A

15s

102
Q

Physical method of Hb estimation

A

rule of three- hct is 3x the hgb value

103
Q

chemical method for iron content. hb iron content= 100g of hb= ___g of Fe++

A

0.347g

104
Q

refers to the instrument used to determine packed cell volune

A

hematocrit

105
Q

ration of RBC with the plasma

A

packed cell volume

106
Q

Hematocrit is reported as __ or __

A

percentage or liters per liter

107
Q

hematocrit normal value. at birth

A

45-60%

108
Q

hematocrit normal value. women

A

36-48%

109
Q

hematocrit normal value. men

A

40-55%

110
Q

hematocrit decreased in ___

A

anemia

111
Q

hematocrit increased in ___

A

Polycythemia vera

112
Q

methods in hematocrit

A

Macrohematocrit
Microhematocrit

113
Q

this hematocrit method is time consuming, large amount of sample needed, higher amount of trappped plasma

A

Macrohematocrit

114
Q

macrohematocrit equipment

A

wintrobe tube

115
Q

Wintrobe tube length

A

11.5 cm/115m long,
3mm internal bore

116
Q

Wintrobe tube. ESR mark

A

0-1000 mark

117
Q

Wintrobe tube. HCT mark

A

1000-0 mark

118
Q

type of hematocrit method wherein less blood and less time is consumed

A

microhematocrit

119
Q

2 types of microhematocrit

A
  1. red band
  2. blue band
120
Q

type of Microhematocrit that uses spray-dried heparin used for sample without anticoagulant

A

red band

121
Q

type of Microhematocrit that is used in anticoagulated sample

A

blue band

122
Q

microhematocrit tube length

A

7-7.5 cm/70-75mm
bore:1.2mm

123
Q

clay sealed equipment of microhematocrit

A

microhematocrit tube

124
Q

microhematocrit tube is centrifuged at 10,000-15,000xg RCF for ___ mins

A

5 mins

125
Q

sources of error in microhematocrit. falsely decreased

A

incomplete sealing
overanticoagulation

126
Q

sources of error in microhematocrit. falsely increased

A

inadequate centrifugation
left standing for several mins

127
Q

this problem occurs only in manual procedure. small amount of plasma remains in PCV after centrifugation (hematocrit)

A

trapped plasma

128
Q

Trapped plasma is seen in ?

A

Macrocytic anemia
hypochromic anemia
spherocytosis
thalassemia
sickle cell anemia

129
Q

test to detect and monitor the course of inflammatory conditions such as rheumatoid arthritis, infx, or certain malignancies

A

Estimated Sedimentation Rate

130
Q

what is this test? Principle: anticoagulated blood allows to settle toward the bottom of the tube. the distance in mm that the RBCs fall in 1 hour

A

Estimated sedimentation rate

131
Q

formation that increase RBC mass in ESR

A

rouleaux formation