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Hema Lec 1 > Chapter 9 > Flashcards

Chapter 9 Flashcards

0
Q

Hereditary deficiency of nearly every enzyme in glycolysis and results to shortened RBC survival

A

Hereditary nonspherocytic hemolytic anemia

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1
Q

2 main functions of RBC

A
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2
Q

Transmembranous cation gradient concentration proteins

A

Cation pumps

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3
Q

Concentrations of pumps

A

⬆️ IC K+ , ⬇️ EC K+
⬇️ IC Na+ , ⬆️ EC Na+
⏬ IC Ca+2 , ⏫ EC Ca+2

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4
Q

Energy-producing enzymes degrade over time, the pumps fail, na, ca, water flow inside the RBC leading to

A

Swelling and destruction

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5
Q

Requires glucose to generate ATP (greatest source of energy in RBC)

A

EMP - anaerobic glycolysis

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6
Q

Consumption of 2 ATP/ glucose molecule
Generation of 4 ATP/ glucose molecule
NET GAIN=?

A

2 molecules of ATP

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7
Q

Final product of phase 1 glycolysis

A

G3P

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8
Q

Rate-limiting in steady state anaerobic glycolysis

A
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9
Q

Product of 2nd phase glycolysis

A

3-PG and ATP

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10
Q

Generation products that maximize oxygen delivery from several vantage points

A

Diversions or Shunts

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11
Q

Detoxifies accumulated peroxide to extend functional life span of the RBC

A

HMP or PPP (AEROBIC or OXIDATIVE Glycolysis)

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12
Q

Agent that OXIDIZES Heme Iron (Fe2+), proteins, lipids (thiol grps)

A

Peroxide

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13
Q

Diverts G6P to Pentose Phosphate

A

G6PD

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14
Q

NADP* ⏩ NADPH (reduced)
NADPH ➡️ GSSG ➖ glutathione reductase ➖ ⏩ GSH
GSH ➡️ Peroxide (H2O2) ➖ glutathione peroxidase - ⏩ H2O + O2

A

HMP

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15
Q

Provide the only means for generation of NADPH for GSSG reduction
RBCs become Susceptible to oxidative damage w/o it

A

G6PD

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16
Q

Most common human RBC enzyme deficiency worldwide ⏩ HEREDITARY NONSPHEROCYTIC ANEMIA

A

G6PD Deficiency

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17
Q

Fe2+ ~peroxide ~ ➡️ Fe3+

metHb Reductase Pathway

A

metHemoglobign (metHb)

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18
Q

Returns oxidized iron (Fe3+) ⏩ Fe2+

65% met-hb reducing capacity in RBC

A

MetHb reductase or cytochrome b5 reductase (cytob5r)

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19
Q

1,3-BPG ~bisphosphoglycerate mutase~ ⏩ 2,3-BPG ➡️ competes with 02 for hemoglobin ➡️ O2 released and delivered to the tissues

A

Rapoport-Luebering Pathway

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20
Q

Inhibits the rapoport shunt by inhibiting bisphosphoglycerate mutase and retaining 1,3-BPG in EMP

A

⬇️ pH

⬇️ 3-PG and 2-PG

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21
Q

RBC membrane consists of

A

52% Proteins

40% Lipids

8% Carbs

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22
Q

__% excess surface are compared to __fL sphere

A

40%

90 fL

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23
Q

40% : 90fL allows RBCs to streched up to __x their resting diameter

A

2.5x – “RBC deformability”

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24
Q

5um thick, 100x more elastic than latex membrane, tensile strength greater than steel

A

RBC plasma membrane

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25
Q

RBC deformability depends on

A
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26
Q

Normal MCHC rangein Hb viscosity

A

32%-36%

⬆️Hb concentration, ⬆️ Viscosity

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27
Q

As RBCs age, they lose membrane surface area while retaining Hb. Hb becomes more and more concentrated, destroyed by splenic macrophage

A

Senescence theories

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28
Q

Cholesterol = Phospholipids

A

Lipid portion

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29
Q

Reseal rapidly when membrane is torn

A

Phospholipids

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30
Q

Esterified and largely hydrophobic; resides parallel to the acyl tails; one molecule per phospholipid

A

Cholesterol

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31
Q

Only hydrophilic portion of cholesterol, anchors within the polar head groups

A

Cholesterol’s B hydroxyl group

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32
Q

Confers tensile strength to the lipid bilayer

A

Cholesterol

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33
Q

Deficiency in membrane enzymes that maintain cholesterol conc
Membrane loses tensile strength

A

Acanthocytosis

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34
Q

Phospholipids in outer layer

A

Pc s

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35
Q

Phospholipids in inner layer

A

Ps pe

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36
Q

Membrane-associated enzymes for distribution/position of the phospholipids

A

Flippases, floppases, scramblases

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37
Q

Phospholipid distribution disruption

A
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38
Q

Phosphatodylserine (only - charge) flips out and destroyed by splenic macrophages

A

Senescence theories

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39
Q

liver disease: ⬇️ bile salt conc, ⬇️ membrane cholesterol conc

A

Target Cell appearance

40
Q

Sugar-bearing lipids that make up 5% of external Half of RBC me,brame

A

Glycolipids

41
Q

Glycolipids associate in clumps or _____ and support _______ that extend into the aqueous plasma to help form ______

A

Rafts
Carbohydrate side chains
GLYCOCALYX

42
Q

Layer of carbs whose net negative charge prevents microbial attack + protects RBC from mechanical damage caused by neighboring RBCs to the endothelium

A

Glycocalyx

43
Q

Ex of carbohydrate-based blood groups carfied by glycolipids

A
44
Q

Process when surface carbs + glycolipids = glycocalyx

A

Glycosylatio

45
Q

They serve as as transport and adhesion sites and signaling receptors

A

Glycolipids

46
Q
  • Vesiculation

- process when RBCs adhere to each other and to the vessel walls

A

Fragmentation

47
Q

Process when signaling receptors ➕ plasma ligands ➡️ activation of submembranous G proteins ➡️ initiate various energy-dependent cell activities

A

Signal transduction

48
Q

Trans proteins assmeble into these two macromolecular complexes (named by their respective anchorages)

A
49
Q

These complexes provide RBC membrane “structural integrity” and VERTICAL MEMBRANE STRUCTURE

A

TRANSMEMBRANOUS PROTEINS

50
Q

Anion transport (most abundant trans protein)

A

Band 3

51
Q

Glucose trnsport

A

Glut-1

52
Q

Band 3 and Glut1 support the majority of ____ by virtue od their high copy numbers

A

ABH system carbs determinants

53
Q

Supports peptide-defined MN determinants

A

Glycophorin A

54
Q

Supports Ss determinants

A

Glycophorin B

55
Q

Glyc A + Glyc B=

A

MNSs system

56
Q

System that employs 2multipass transmem lipoproteins and a multipass glycoprotein that each cross the membrane 12 times

A

Rh system

57
Q

Two lipoproteins of Rh system

A

D and CcEe epitopes

58
Q

Necessary for expression of D and CcEe antigens, which localizes near the Rh lipoproteins in the ANKYRIN complex

A

Glycoprotein RhAG

59
Q

Lack of RhAg glycoprotein and associated with morphologic RBC abnormalities

A

Rh-null

60
Q 
Water transporter (trans protein)
-inward flow of water
A

Aquaporin 1

61
Q

Ca2+ transporter (trans protein)

A

Ca2+ ATPase

62
Q

Supports duffy antigens (trans protein)

A

Duffy

63
Q

Supports Gerbich system determinants (trans protein)

A

Glycophorin C

64
Q

Integrin adhesion (trans protein)

A

ICAM4

65
Q

zinc binding endopeptidase, Kell antigens (trans protein)

A

Kell

66
Q

Urea transporter (trans protein)

A

Kidd

67
Q

2 proteins that anchor to ankyrin

A

Band 3

Protein 4.2

68
Q

Backbone bound to Protein 4.1

A 
Dematin
Adducin
Actin
Tropomyosin
Tropomodulin
69
Q
  • found in outer plasma side portion of lipid bilayer

- anchors for 2 surface proteins: DAF (CD55) and MIRL (CD59

A

Phosphatidylinositol (PI)

70
Q

DAF and MIRL float on surface of the membrane as they link to PI through a ____ with multiple sugars

A

Glycan core

71
Q

Links CD55 and CD59 to PI

A

Phosphatidylinositol glycan Class A (PIG-A)

72
Q

PIG-A mutation, ⬇️CD55 and CD59,

Cell susceptible to complement-mediated hemolyisis

A

PNH

73
Q

Numerical naming of 15 proteins

Migration thru gel with velocity MW net charge

A

SDS PAGE

74
Q

Dye to stain BANDS in sds page

A

Coomassie blue

75
Q

Dye to stain GLYCOPHORINS (abundant barc side chain)

A

Periodic acid schiff dye (PAS dye)

76
Q

Links band 3 to protein 4.1

A

Adducin

77
Q

Links glut-1 to protein 4.1

A

Dematin

78
Q

Principal skeletal proteins

A

Alpha and beta spectrins

79
Q

Alpha and beta spectrins assempble to form ____ held by lateral bonds

A

Antiparallel heterodimer

80
Q

COOH end ➖➖➖ NH3 end of other

A

Ntiparallel

81
Q

Provides lateral membrane stability

A

Skeletal proteins / peripheral proteins (spectrins in hexagonal lattice)

82
Q

Secondary structure of both alpha/beta spectrin features

A

Triple helical repeats of 106 amino acids each

83
Q

20 triple helical repeats make up____

A

Alpha spectrin

84
Q

16 triple helical repeats make up____

A

Beta spectrin

85
Q

14-16 monomers whose length is regulated by TROPOMYOSIN

A

Actin

86
Q

Cap the ends of ACTIN

A

Adducin

Tropomodulin

87
Q

Stabilizes actin (?)

A

Dematin

88
Q

Triple helix held by

A

Actin

4.1

89
Q

⏬vertical anchorages, Lipid membrane peels of in small blebs called

A

Vesicles

90
Q

Autosomal dominant mutations affecting SPECTRIN DIMER TO DIMER LATERAL BONDS or SPECTRIN-ANKYRIN-4.1 junction

A

Hereditary elliptocytosis or OVALOCYTOSIS

91
Q

Membrane fails to rebound from deformation ➡️ RBCs elongate to form _____ ➡️ mild to severe hemolytic ANEMIA

A

ELLIPTOCYTES

92
Q

Rbc membrane permeable to:

A

H20
HCO3’
Cl’

93
Q

Regulate conc of Na and K

A

ATPase- dependent cation pumps

94
Q

IC:EC ratio

A

Na+ 1:12

K+ 25:1

95
Q

Pump mechanism damage permits the influx of ____. Cell swells ➡️ spheroid ➡️ ruptures ➡️ Hb spilled called ____

A

NA+ and H20

COLLOID OSMOTIC HEMOLYSIS

96
Q

Extrudes calcium

IC: 5-10 umol/L

A

Ca2+ ATPase

97
Q

A cytoplasmic Ca2+ binding protein, CONTROLS THE FUNCTION OF Ca2+ ATPase

A

Calmodulin

98
Q

Increased Cation permeability

Sickle Hb deforms cell ➡️ HIGH IC Na, K, Ca ➡️ hemolysis

A

Sickle cell disease

Hema Lec 1 (1 decks)

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