01 LEC: Erythrocyte metabolism & membrane structure Flashcards

1
Q

Glucose enters the RBC by facilitated diffusion via the transmembrane protein ___

A

Glut-1

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2
Q

The ___ is an anaerobic pathway that generates ATP

A

Embden Meyerhof Pathway

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3
Q

The enzyme deficiency present for defective Embden Meyerhof Pathway

A

Pyruvate kinase deficiency

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4
Q

The hexose monophosphate shunt is also called

A

Pentose phosphate shunt

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5
Q

The hexose monophosphate shunt provides ___ and ___

A

reduced glutathione and NADPH

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6
Q

Enzyme deficiency for defective hexose monophosphate shunt

A

glucose-6-phosphate dehydrogenase deficiency

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7
Q

G6PD deficiency causes ___ ___ anemia

A

episodic hemolytic

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8
Q

In G6PD, the denatured hgb produces ___

A

Heinz bodies

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9
Q

Where can we see presence of bite cells/pitted cells?

A

G6PD deficiency

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10
Q

T/F: bite cells can be seen using wright stain

A

T

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11
Q

RBC components that can be seen through supravital staining

A

Reticulocyte
Heinz bodies
Hemoglobin H

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12
Q

The methemoglobin reductase pathway is also called ___

A

cytochrome b5 reductase

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13
Q

The methemoglobin reductase pathway is able to ___ ___ once it forms

A

Reduce methemoglobin

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14
Q

T/F: NADPH is more efficient in reducing methemoglobin than cytochrome b5

A

F

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15
Q

This pathway generates 2,3-bisphosphoglycerate

A

Rapoport-Luebering pathway

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16
Q

Importance of 2,3-DPG/BPG in the erythrocyte metabolism

A

Expels out O2 from the hemoglobin

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17
Q

The binding of 2,3-DPG/BPG shifts the hemoglobin-oxygen dissociation curve to the ___, which enhances delivery of oxygen to the tissues

A

right

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18
Q

RBC membrane components

A

CHO 10%
Protein 50%
Lipid 40%

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19
Q

RBC membrane phospholipids are ___ distributed

A

asymmetirically

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20
Q

___ and ___ predominate in the outer layer of RBC membrane

A

phosphotidylcholine
sphingomyelin

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21
Q

___ and ___ predominate in the inner layer of RBC membrane

A

phosphatidylserine
phosphatidylethanolamine

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22
Q

This is described as regularly spaced projections present in a cell

A

echinocyte/burr cell

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23
Q

This is described as large, irregular projections present in a cell

A

acanthocytosis/spur cell

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24
Q

___ and ___ are associated with abnormalities in the concentration or distribution of membrane cholesterol and phospholipids

A

acanthocytosis and target cells

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25
Q

transmembrane and cytoskeletal proteins make up ___ of the half of the RBC membrane structure by mass

A

52%

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26
Q

This serve many functions, including transport sites, adhesion sites, and signaling receptors

A

transmembrane proteins

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27
Q

Identify the transmembrane protein:

water transporter, colton antigen

A

aquaporin 1 (AQP1)

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28
Q

Identify the transmembrane protein:

anion transporter, location of ABH antigens

A

Band 3 (SLC4A1)

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29
Q

Identify the transmembrane protein:

G protein-coupled receptor, chemokine receptor, Duffy antigens, receptor for malarial parasites

A

Duffy (FY, DARC, ACKR1)

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30
Q

Identify the transmembrane protein:

Glucose transporter, location of ABH blood group antigens

A

Glut-1 (SLC2A1)

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31
Q

Identify the transmembrane protein:

Sialic acid transporter, location of MN blood group antigens

A

Glycophorin A (GYPA)

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32
Q

Identify the transmembrane protein:

Sialic acid transporter, location of Ss blood group antigens

A

Glycophorin B (GYPB)

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33
Q

Identify the transmembrane protein:

Sialic acid transporter, location of Gerbich system antigens

A

Glycophorin C (GYPC)

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34
Q

Identify the transmembrane protein:

Zn binding endopeptidase

A

Kell (KEL)

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35
Q

Identify the transmembrane protein:

Urea transporter

A

Kidd (SLC14A1)

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36
Q

Identify the transmembrane protein:

D and CcEe antigens; stabilizes band 3 and Rh macrocomplexes

A

Rh (RHCE, RHD)

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37
Q

Located in membrane macromolecular complexes that serve as transporters, structural components, enzymes, receptors, and adhesion molecules

A

Blood group antigens

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38
Q

___ and ___ support the majority of ABH system

A

Band 3 & Glut-1

39
Q

Glycophorins C & D carry the ___

A

Gerbich system antigens

40
Q

Loss of the RhAG glycoprotein (Rh-null) prevents expression of both the D and CcEe antigens and is associated with RBC morphologic abnormalities such as ___

A

stomatocytosis

41
Q

A few copies of the ___ ___ reside in the outer, plasma-side layer of the membrane, which serves as a base on which a glycan core of sugar molecules is synthesized

A

phospholipid phosphatidylinositol (PI)

42
Q

PI serves as a base for a glycan core to synthesize ___

A

glycosylphosphatidylinositol (GPI) anchor

43
Q

They protect the RBC membrane from lysis by complement

A

GPI anchored proteins

44
Q

The GPI anchored proteins include:

A

Decay-accelerating factor (CD55)
Membrane Inhibitor of Reactive Lysis (MIRL or CD59)

45
Q

An acquired mutation of PIGA genes affect the cells and make them susceptible to ___ ___

A

complement-mediated hemolysis

46
Q

The principal cytoskeletal proteins are the filamentous ___ and ___

A

a-spectrin and b-spectrin

47
Q

They arise from defects in spectrin or proteins forming the ankyrin complex that provide vertical support for the membrane

A

hereditary spherocytsosis

48
Q

They are due to defects in cytoskeletal proteins that provide horizontal support for the membrane

A

hereditary elliptocytosis

49
Q

Identify the cytoskeletal protein:

filamentous antiparallel heterodimer, primary cytoskeletal proteins

A

a-spectrin
b-spectrin

50
Q

Identify the cytoskeletal protein:

Anchors band 3, protein 4.2, and other proteins

A

Ankyrin

51
Q

Identify the cytoskeletal protein:

Anchors the actin junctional complex to spectrin tetramers, RBC cytoskeleton shape

A

Protein 4.1

52
Q

Identify the cytoskeletal protein:

Part of ankyrin complex, ATP binding protein

A

Protein 4.2

53
Q

Heme is composed of ___ and ___

A

Protoporphyrin IX and Fe2+

54
Q

Heme synthesis begins in the ___ with the formation of ___

A

mitochondria
ALA

55
Q

___ portion gives RBC their characteristic red color

A

heme

56
Q

Chromosome __ codes for Alpha and Zeta

A

16 (short arm)

57
Q

Chromosome __ codes for Beta, Gamma, Delta, and Epsilon

A

11 (short arm)

58
Q

The primary hemoglobin until 6 months of age

A

HgbF

59
Q

This factor binds in the B-gene promoter region

A

Kruppel-like factor 1 (KLF-1)

60
Q

T/F: during oxygenation, each one of the four heme iron atoms in a hemoglobin molecule irreversibly bind one O2 molecule

A

F (reversible)

61
Q

Approximately ___ of oxygen is bound by each gram of hemoglobin

A

1.34 mL

62
Q

Normally, a PO2 of approximately ___ results in 50% O2 saturation

A

27 mmHg

63
Q

A shift in the curve because of a change in pH

A

Bohr effect

64
Q

During __ state, there is low affinity to oxygen and it is less likely to bind to O2 molecules

A

tensed (right shift)

65
Q

During __ state, the blood is in deoxygenated form

A

tensed (right shift)

66
Q

The __ state is stabilized by a network of salt bridges and hydrogen bonds between subunits

A

tensed (right shift)

67
Q

The tensed state is more stable in condition of __ pH and __ conc of 2,3-DPG

A

low, high

68
Q

During __ state, hemoglobin has a higher affinity for oxygen

A

relaxed (left shit)

69
Q

During __ state, the hemoglobin is typically in its oxygenated form

A

relaxed (left shift)

70
Q

During relaxed state, binding of O2 disrupts the ___ and ___ that stabilized the t state

A

salt bridges
hydrogen bonds

71
Q

This is formed by reversible oxidation of heme iron to the ferric state

A

methemoglobin

72
Q

Occurs after exposure to exogenous oxidants such as nitrites, primaquine, dapsone, benzocaine

A

Acquired/toxic methemoglobin

73
Q

30-50% methemoglobin, structurally abnormal polypeptide chain that favors the oxidized ferric form of iron and prevent its reduction

A

M hemoglobin/ HbM

74
Q

Levels of methemoglobin and its interpretation

A

<25% asymptomatic
>30% cyanosis, hypoxia (needs IV methylene blue)
>50% coma and death

75
Q

Methemoglobin causes ___ discoloration of blood

A

chocolate brown

76
Q

Methemoglobin is detected at ___ nm spectral absorption

A

630 nm

77
Q

The treatment for methemoglobin is ___

A

methylene blue

78
Q

Irreversible oxidation of hgb by materials containing sulfur or exposure to sulfur chemicals

A

sulfhemoglobin

79
Q

Addition of sulfur atom to pyrrole ring results to ___ pigment

A

greenish

80
Q

Sulfhemoglobin is also reported in px with severe constipation in cases of bacteremia due to ___

A

C. perfringens

81
Q

T/F: Sulfhemoglobin is reversible and can be converted back to adult hemoglobin

A

F (persist for the life of the cell :(()

82
Q

Sulfhemoglobin causes ___ discoloration of blood

A

mauve lavender

83
Q

Methemoglobin is detected at ___ nm spectral absorption

A

630

84
Q

Cyanmethemoglobin is detected at ___ nm spectral absorption

A

540

85
Q

Cyanmethemoglobin measures all forms of hgb except ___

A

sulfhemoglobin

86
Q

Combination of CO with heme iron

A

Carboxyhemoglobin (COHb)

87
Q

In COHb, the affinity of CO is ___x that of oxygen

A

240 (200)

88
Q

__ has been termed as the silent killer, because it is an odorless and colorless gas, and victims may quickly become hypoxic

A

CO

89
Q

Diagnosis of CO poisoning is made if the COHb levels is ___ in nonsmokers and ___ in smokers

A

3%
10%

90
Q

How many % til COHb symptoms appear?

A

20-30%

91
Q

How many % til COHb cause coma, seizure, or death?

A

> 40%

92
Q

COHb causes ___ discoloration of blood

A

cherry red color (skin also !)

93
Q

COHb is detected at __ nm spectral absorption

A

540

94
Q

Treatment for COHb

A

hyperbaric oxygen therapy