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HEMA LEC > Erythrocyte Metabolism & Membrane Structure, Hemoglobin Metabolism > Flashcards

Erythrocyte Metabolism & Membrane Structure, Hemoglobin Metabolism Flashcards

handout ni sir

1
Q

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

A

Glut-1

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2
Q

the essential enzyme in HMP

A

glucose-6-phosphate dehydrogenase

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3
Q

end products of EMP (2)

A
  • pyruvate
  • lactate
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4
Q

T/F:

EMP does not generate atp

A

False

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5
Q

In EMP, what is the enzyme deficiency?

clue: isipin mo product ng EMP

A

Pyruvate Kinase Deficiency

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6
Q

Pyruvate Kinase Deficiency would produce what type of red cell?

A

Echinocyte/crenated/Burr cell

same lang yan

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7
Q

Give a function of RBC that does not need ATP

A

Oxygen Transport

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8
Q

how many atp consumed in glycolysis?

A

2 ATP

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9
Q

Ferrous or Ferric

which forms methemoglobin?

A

Ferric Iron

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10
Q

characteristics of ferric iron

A
  • forms methemoglobin/hemiglobin
  • can’t bind O2; makes hemoglobin dysfunctional
  • reversible
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11
Q

sodium-potassium ATPase pump forms what rbc?

A

stomatocytes

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12
Q

RBC with mouth-like appearance central pallor

A

stomatocytes

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13
Q

PATHWAY

detoxifies peroxide (H2O2)

A

HMP Shunt

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14
Q

enzyme deficiency in HMP shunt

A

G6PD deficiency

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15
Q

G6PD deficiency will cause.. (3)

A
  • denatured Hemoglobin (Heinz bodies)
  • supravital stain (RHH)
  • bite cells
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16
Q

HMP Shunt

what can be seen with supravital stain

RHH

A
  • reticulocytes
  • Heinz bodies
  • Hemoglobin H
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17
Q

products of HMP shunt

A
  • reduced glutathione (GSSG)
  • NADPH
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18
Q

MRP is also called..

A

Cytochrome B5 Reductase

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19
Q

Methemoglobin Reductase Pathway can convert _ back to _

A

ferric back to ferrous

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20
Q

enzyme deficiency in MRP

A

cytochrome b5 deficiency

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21
Q

If you are deficient in Cytochrome B5, your blood color is..

A

chocolate brown

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22
Q

pathway

generates 2,3-diphosphoglycerate

A

Rapoport-Luebering Pathway

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23
Q

This binding shifts the** Hgb-O2 dissociation curve** to the right, which enhances the delivery of oxygen to tissues

A

Rapoport-Luebering Pathway

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24
Q

high or low

if 2,3-BPG is high, delivery of O2 to tissues is _

A

high

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25
Q

RBC membrane is composed of: (3)

A
  • protein (50%)
  • lipid (40%)
  • CHO (10%)
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26
Q

RBC membrane

predominate in the outer layer (2)

A
  • phosphatidylcholine
  • sphingomyelin
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27
Q

RBC membrane

predominate in the inner layer (2)

A
  • phosphatidylserine
  • phosphatidylethanolemine
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28
Q

_ and _ constitute the principal RBC membrane structure

A

cholesterol and phospholipids

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29
Q

_ are associated with abnormalities in the conc or distribution of membrane cholesterol and phospholipids

A

acanthocytosis & target cells

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30
Q

other name for acquired acanthocytosis

A

Spur cell anemia

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31
Q

this is an alcoholic chronic liver disease

A

acquired acanthocytosis (spur cell anemia)

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32
Q

Transmembrane Proteins

2 major macromolecular complexes

A
  • ankyrin complex
  • actin junctional complex
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33
Q

provide support and to connect the lipid bilayer to the underlying cytoskeleton to maintain membrane integrity

A

Transmembrane proteins

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34
Q

Transmembrane proteins

water transporter

A

Aquaporin 1

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35
Q

Transmembrane proteins

Anion transporter

A

Band 3

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36
Q

which is not a transmembrane protein?

  • Band 3
  • Glycophorin B
  • Ankyrin
  • ICAM-4
A

Ankyrin

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37
Q

Transmembrane or Skeletal Protein?

Protein 4.1

A

Skele

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38
Q

Transmembrane or Skeletal Protein?

RhAG

A

TM

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39
Q

Transmembrane or Skeletal Protein?

Duffy

A

TM

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40
Q

Transmembrane or Skeletal Protein?

Glut-1

A

TM

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41
Q

Transmembrane or Skeletal Protein?

Kell

A

TM

42
Q

Transmembrane or Skeletal Protein?

a-spectrin

A

skele

43
Q

Transmembrane or Skeletal Protein?

adducin

A

skele

44
Q

Transmembrane or Skeletal Protein?

G3PD

A

skele

45
Q

Skeletal Protein

anchors the actin junctional complex to spectrin tetramers

A

Protein 4.1

46
Q

Skeletal protein

filamentous antiparallel heterodimer

A

a-spectrin

47
Q

Transmembrane Proteins

_ and _ support the majority of the ABH system

A
  • Band 3
  • Glut-1
48
Q

Transmembrane Proteins

carries the peptide-defined M and N determinants

A

Glycophorin A

49
Q

Transmembrane Proteins

carried the **Ss **determinants

A

Glycophorin B

50
Q

Transmembrane Proteins

_ and _ together comprise the MNS system

A
  • Glycophorin A
  • Glycophorin B
51
Q

Transmembrane Proteins

Glycophorin C and D carry the..

A

Gerbich System Antigens

52
Q

RH null results in _

A

stomatocytes/stomatocytosis

53
Q

PI serves as a base on which a glycan core of sugar molecule is synthesized, forming the

A

glycosylphosphatidylinositol

54
Q

Enumerate

GPI anchored proteins (2)

A
  • Decay Accelerating Factor (CD55)
  • Membrane Inhibitor of Reactive Lysis (CD59)
55
Q

if there are no CD55 and CD59, the person is prone to _

A

complement mediated hemolysis

aka Paroxysmal Nocturnal Hemoglobinuria

56
Q

what is used to detect Paroxysmal Nocturnal Hemoglobinuria?

A

flow cytometry

57
Q

An acquired mutation in this gene affects the cells’ ability to synthesize the GPI anchor

without it, the cell memb becomes CD55 and CD59 deficient

A

Phosphatidylinositol Glycan Anchor Biosynthesis Class A** (PIGA)**

58
Q

Principal cytoskeletal proteins (2)

A
  • alpha-spectrin
  • beta-spectrin
59
Q

cytoskeletal proteins

_ arises in the defects of vertical support

A

Hereditary spherocytosis

60
Q

cytoskeletal proteins

_ is due to defects in horizontal support

A

Hereditary elliptocytosis

61
Q

_ synthesis occurs first and last in the _

A

heme synthesis; mitochondrion

62
Q

enzyme that binds iron to protoporphyrin to form heme

A

Ferrochelatase (Fe 2+) aka Heme synthase

63
Q

sensitive to light; burgundy urine

A

porphyria

64
Q

how many heme groups are attached to four globin chains?

A

4 heme grps

65
Q

what RBC stage are heme & globin components first synthesized?

A

pronormoblast

66
Q

Protoporphyrin IX + Fe2+ = …

A

Heme

67
Q

what gives RBC its red color?

A

heme

68
Q

Hemoglobinn is first present in the _

A

polychromatic normoblast

69
Q

Globin

2 identical pairs of unlike polypeptide chains

A

2 alpha, 2 non alpha

70
Q

chromosome

codes for Alpha and Zeta

A

Chromosome 16 (short arm)

71
Q

chromosome

codes for delta and gamma

A

Chromosome 11 (short arm)

beta, gama, delta, epsilon

72
Q

T/F:

alpha genes are 2 copies per chromatid

A

True

so 4 alpha genes (2 from mom, 2 from dad)

73
Q

globin chain of Gower 1

A

2 zeta + 2 epsilon

74
Q

globin chain of Gower 2

A

2 alpha + 2 epsilon

75
Q

globin chain of portland

A

2 zeta + 2 gamma

76
Q

Hgb F is primary hgb until _ months

A

6 mos of age

77
Q

what binds in the beta gene promoter region?

A

Kruppel-like Factor 1** (KLF-1)**

78
Q

approximately _ mL of oxygen is bound by each gram of Hgb

A

1.34 mL

79
Q

Left or Right Shift

Low delivery of O2 to tissues

A

Left

80
Q

Left or Right Shift

reduced P50 / <27mmHg

A

Left

81
Q

Left or Right Shift

increased P50 / > 27mmHg

A

Right

82
Q

Left or Right Shift

alkalosis

A

Left

83
Q

Left or Right Shift

high body temp

A

Right

84
Q

Left or Right Shift

relaxed state

A

Left

85
Q

Left or Right Shift

acidosis

A

Right

acidosis = low pH

86
Q

a shift in the curve due to change in PH

A

Bohr Effect

87
Q

state

low affinity for oxygen

A

Tensed state

shift to right

88
Q

state

deoxygenated

A

Tensed state

89
Q

state

High affinity for oxygen

A

Relaxed state

90
Q

Enumerate

dysfunctional hemoglobins (3)

A
  • methemoglobinemia
  • sulfhemoglobinemia
  • carboxyhemoglobinemia
91
Q

enumerate

blood color of dysfunctional hemoglobin (3)

A
  • methemoglobinemia: chocolate brown
  • sulfhemoglobinemia: mauve lavender
  • carboxyhemoglobinemia: cherry red
92
Q

dysf hemoglobin

which has mauve lavender blood?

A

sulfhemoglobinemia

93
Q

Enumerate

Methemoglobin (3 types)

A
  • acquired/toxic methemoglobinemia
  • M Hemoglobin
  • Cytochrome B5 Reductase Deficiency
94
Q

treatment for methemoglobin

A

methylene blue

injected intravenously; reducing agent

95
Q

monoxide or dioxide?

carboxyhemoglobin is a combination of carbon _ with heme iron

A

monoxide

96
Q

which dysfunctional hemoglobin is irreversible?

A

Sulfhemoglobin

97
Q

color/pigment in sulfhemoglobin

A

greenish pigment

98
Q

cyanmethemoglobin method measures all forms of Hgb except _

A

sulfhemoglobin

99
Q

diagnosis of carbon monoxide poisoning in smorkers

A

> 10%

if nonsmoker, > 3%

100
Q

dysfunctional hemoglobin

detected at 630 nm

A
  • methemoglobin
  • sulfhemoglobin
101
Q

dysfunctional hmoglobin

detected at 540 nm

A

carboxyhemoglobinemia

102
Q

treatment for carboxyhemoglobinemia

A

hyperbaric oxygen therapy

HEMA LEC (1 decks)

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