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Hema - Midterms > Hemoglobin > Flashcards

Hemoglobin Flashcards

1
Q

Components of hemoglobin

A

4 heme groups and 2 heterogenous pairs of polypeptide chains

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2
Q

Concentration of hemoglobin in rbc

A

34 g/dL

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3
Q

Hemoglobin carries how many ferrous ions

A

2

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4
Q

1 g of hemoglobin can carry how many mL of oxygen?

A

1.34 mL

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5
Q

It is the main respiratory pigment and main component seen in RBC

A

Hemoglobin

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6
Q

Functions of hemoglobin

A
  • transport O2 from lungs to tissue
  • transport CO2 from tissue to lungs
  • contribute to acid base balance by binding and releasing hydrogen ions
  • modulates vascular dilation by transporting NO
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7
Q

True or false

One heme group is embedded in each polypeptide chain

A

True

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8
Q

A qualitative disorder that pertains to the variation / mutation within amino acid sequence

A

Hemoglobinopathies

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9
Q

A quantitative disorder that pertains to a gene defect that don’t code for a globin chain

A

Thalassemia

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10
Q

Range of amino acids that a globin chain contains

A

141 - 146

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11
Q

Each globin chain is divided into

A

8 helices and 7 non helical segments

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12
Q

True or false

The hemoglobin molecule can be described by its protein stucture

A

True

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13
Q

A predominant adult Hb that is composed of 2 alpha and 2 beta globin chain

A

Hb A

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14
Q

Globin chains loop to form a ________ for heme

A

Cleft pocket

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15
Q

Shape of a hemoglobin molecule

A

Spherical

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16
Q

Hemoglobin can carry up to how many O2,?

A

4

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17
Q

Hemoglobin that has a sugar attached js known as

A

Glycated

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18
Q

True or false

Small percentage of Hb F is glycated

A

False

Hb A

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19
Q

Chain arrangements in helices and non helices

A

Secondary structure

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20
Q

Combination of amino acid sequence and polypeptide chains

A

Primary structure

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21
Q

Arrangement of helices in a pretzel-like configuration

A

Tertiary structure

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22
Q

Heme biosynthesis occurs in the

A

Mitochondria and cytoplasm of rbc bone marrow precursor

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23
Q

True or false

The loss of mitochondria and ribosomes results to the cessation of hemoglobin synthesis

A

True

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24
Q

Give the enzymes responsible for heme biosynthesis in order

A 
ALA synthase
ALA dehydratase
PBG deaminase
Uroporphyrinogen III cosynthase
Uroporphyrinogen III decarboxylase
Corpophyrinigen oxidase
Protoporphyrinogen IX oxidase
Ferrochelatase
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25
Q

It is also called the heme synthase

A

Ferrochelatase

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26
Q

Ion added to the heme synthase to make heme

A

Ferrous ion

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27
Q

Where does heme biosynthesis occurs

A

Mitochondria and cytoplasm

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28
Q

Transferrin bound to transferrin receptor are brought in the cell in a form of

A

Endosome

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29
Q

Alpha and zeta globin chain can be found in what chromosome

A

Chromosome 16

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30
Q

Chromosome 11 is associated with what globin chains?

A

Beta gamma delta epsilon

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31
Q

Production of globin biosynthesis occurs in

A

Eryhthroid precursor

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32
Q

Acute intermittent porphyria is associated with this enzyme

A

PBG deaminase

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33
Q

Ferrochelatase can cause what disease?

A

Erythropoietin protoporphyria

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34
Q

What disease is assoc with coproporphyrinogen?

A

Hereditary coproporphyria

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35
Q

Uroporphyrinogen III decarboxylase can be assoc with what disease

A

Porphyria cutanea tarda

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36
Q

Deficiency in specific enzymes involved in heme synthsesis

A

Porphyria

37
Q

Where does the heme attach in the globin chains?

A

Cleft pocket

38
Q

Disease with protoporphyrinogen oxidase and the product it produce

A

Variegate porphyria

Protoporphyrin IX

39
Q

Enumerate in order the the chain with from highest affinity to lowest affinity

A

Alpha gamma delta

40
Q

Combination of 2 heterodimer

A

Tetramer

41
Q

Completes the hemoglobin molecule

A

Tetramer

42
Q

Composition of heterodimer

A

Globin chain and heme molecule

43
Q

Globin chains of Hb A

A

2 alpha and 2 beta

44
Q

Globin chains that hold the dimers in stable

A

Alpha 1 - beta 1 and alpha 2 - beta 2 bonds

45
Q

Alpha 1 - beta 2 and alpha 2 - beta 1 bond is important for

A

Stability of the quaternary structure

46
Q

Small portion of Hb A that is glycated

A

Hb A1c

47
Q

Disease assoc. with glycated hemoglobin

A

Uncontrolled diabetes mellitus

48
Q

2 alpha and 2 delta globin chains comprise what Hb

A

Hb A2

49
Q

F or A/F cells are what?

A

Small portion of RBCs with Hb F

50
Q

Embryonic hemoglobins:

A

Gower 1, Gower 2, Portland

51
Q

Predominant Hb in the 2nd and 3rd trimester

A

Hb F

52
Q

Hb in adulthood

A

Hb A Hb F Hb A2

53
Q

Hydroxymethlybilane sythase is acted on what reaction

A

PBG to hydroxymethylbilane

54
Q

<3.5% of Total Hb is what Hb?

A

Hb A2

55
Q

Embryonic hemoglobin is formed by the combination of these globin chains

A

Alpha and gamma globin chains and zeta and epsilon globin chains

56
Q

Heme inhibits

A

ALA synthase
Ferrochelatase
ALA dehydratase
PBG deaminase

57
Q

Ferrochelatase is inhibited by

A

Heme and protoporphyrin IX

58
Q

Abnormal production if alpha chain

A

Alpha thalassemia

59
Q

Excess globin chains, protoporphyrin IX and iron can cause

A

Damage to the cell leading to reduce life span

60
Q

Regulator role in heme synthesis

A

Ferrochelatase

61
Q

Key regulating step

A

Glycine + succinyl coenzyme a —> ALA

62
Q

DNA Sequences in globin production

A

Cis acting promoters, enhancers, silencers

63
Q

Binds to DNA or one another to promote or suppress transcription

A

Trans acting factor

64
Q

Inhibition of transcription of a particular globin gene requires:

A 
Promoter DNA sequences
Krüppel like factor 1
TAL 1
GATA1 
LDB 1
Ikaros
P45 NF E2
Locus control region
65
Q

Key regulator in gamma beta switching

A

Krüppel like factor 1

66
Q

Repressors of gamma globin gene transcription

A

BCL11A

MYB

67
Q

Ferric oxidation product of heme

A

Hemin

68
Q

Components of Hb that reduce RBC survival when in excess

A

Unpaired chains
Protoporphyrin
Iron

69
Q

Controls the rate of globin synthesis

A

Hemin

70
Q

Absence of hemin will lead to

A

Inhibitor accumulation

71
Q

1 gram hemoglobin eqauls to

A

1.34 ml of oxygen

72
Q

1 mRNA =

A

1 globin

73
Q

Tissue hypoxia occurs in

A

Insufficient hemoglobin

Defect of hemoglobin in carrying oxygen

74
Q

Tissue hypoxia is detected by

A

Peritubular cells in kidney

75
Q

High or low affinity for oxygen
To transport oxygen:
To unload oxygen:

A

High

Low

76
Q

Hahahahaha

A

Funny

77
Q

Graphic expression of hemoglobin

A

Oxygen dissociation curve of hemoglobin

78
Q

The curve in oxygen dissociation curve is termed

A

Sigmoidal

79
Q

Hugh

A

Jackman

80
Q

High hemoglobin affinity for oxygen at _______ oxygen tension

A

High

81
Q

Shift in curve due to change in hydrogen ion (pH change)

A

Bohr effect

82
Q 
Shift to left or right
Lower oxygen affinity:
High pH
Low body temp
Presence of Hb variants with high affinity for oxygen
Acidosis
A 
Right
Left 
Left 
Left
Right
83
Q

Tense confirmation has oxygenated or deoxygenated hemoglobin?

A

Deoxygenated

84
Q

R state or relax state hax oxygenated or deoxygenated hemoglobin?

A

Oxygenated

85
Q

MW of myoglobin

A

17,000 Dalton

86
Q

Myoglobin release in plasma is what disease

A

Rhabdomyolysis

87
Q

Facilitates the reaction of producing carbonic acid

A

Carbonic anhydrase

88
Q

Production of carbonic acid occurs in

A

RBC

89
Q

Ion that diffuses in to cell form plasma to maintain neutrality across membrane

A

Chloride

Hema - Midterms (1 decks)

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