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Hema Lec 1 > Chapter 9 > Flashcards

Chapter 9 Flashcards

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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
5um thick, 100x more elastic than latex membrane, tensile strength greater than steel
RBC plasma membrane
25
RBC deformability depends on
26
Normal MCHC rangein Hb viscosity
32%-36% | ⬆️Hb concentration, ⬆️ Viscosity
27
As RBCs age, they lose membrane surface area while retaining Hb. Hb becomes more and more concentrated, destroyed by splenic macrophage
Senescence theories
28
Cholesterol = Phospholipids
Lipid portion
29
Reseal rapidly when membrane is torn
Phospholipids
30
Esterified and largely hydrophobic; resides parallel to the acyl tails; one molecule per phospholipid
Cholesterol
31
Only hydrophilic portion of cholesterol, anchors within the polar head groups
Cholesterol's B hydroxyl group
32
Confers tensile strength to the lipid bilayer
Cholesterol
33
Deficiency in membrane enzymes that maintain cholesterol conc Membrane loses tensile strength
Acanthocytosis
34
Phospholipids in outer layer
Pc s
35
Phospholipids in inner layer
Ps pe
36
Membrane-associated enzymes for distribution/position of the phospholipids
Flippases, floppases, scramblases
37
Phospholipid distribution disruption
38
Phosphatodylserine (only - charge) flips out and destroyed by splenic macrophages
Senescence theories
39
liver disease: ⬇️ bile salt conc, ⬇️ membrane cholesterol conc
Target Cell appearance
40
Sugar-bearing lipids that make up 5% of external Half of RBC me,brame
Glycolipids
41
Glycolipids associate in clumps or _____ and support _______ that extend into the aqueous plasma to help form ______
Rafts Carbohydrate side chains GLYCOCALYX
42
Layer of carbs whose net negative charge prevents microbial attack + protects RBC from mechanical damage caused by neighboring RBCs to the endothelium
Glycocalyx
43
Ex of carbohydrate-based blood groups carfied by glycolipids
44
Process when surface carbs + glycolipids = glycocalyx
Glycosylatio
45
They serve as as transport and adhesion sites and signaling receptors
Glycolipids
46
- Vesiculation | - process when RBCs adhere to each other and to the vessel walls
Fragmentation
47
Process when signaling receptors ➕ plasma ligands ➡️ activation of submembranous G proteins ➡️ initiate various energy-dependent cell activities
Signal transduction
48
Trans proteins assmeble into these two macromolecular complexes (named by their respective anchorages)
49
These complexes provide RBC membrane "structural integrity" and VERTICAL MEMBRANE STRUCTURE
TRANSMEMBRANOUS PROTEINS
50
Anion transport (most abundant trans protein)
Band 3
51
Glucose trnsport
Glut-1
52
Band 3 and Glut1 support the majority of ____ by virtue od their high copy numbers
ABH system carbs determinants
53
Supports peptide-defined MN determinants
Glycophorin A
54
Supports Ss determinants
Glycophorin B
55
Glyc A + Glyc B=
MNSs system
56
System that employs 2multipass transmem lipoproteins and a multipass glycoprotein that each cross the membrane 12 times
Rh system
57
Two lipoproteins of Rh system
D and CcEe epitopes
58
Necessary for expression of D and CcEe antigens, which localizes near the Rh lipoproteins in the ANKYRIN complex
Glycoprotein RhAG
59
Lack of RhAg glycoprotein and associated with morphologic RBC abnormalities
Rh-null
60
``` Water transporter (trans protein) -inward flow of water ```
Aquaporin 1
61
Ca2+ transporter (trans protein)
Ca2+ ATPase
62
Supports duffy antigens (trans protein)
Duffy
63
Supports Gerbich system determinants (trans protein)
Glycophorin C
64
Integrin adhesion (trans protein)
ICAM4
65
zinc binding endopeptidase, Kell antigens (trans protein)
Kell
66
Urea transporter (trans protein)
Kidd
67
2 proteins that anchor to ankyrin
Band 3 | Protein 4.2
68
Backbone bound to Protein 4.1
``` Dematin Adducin Actin Tropomyosin Tropomodulin ```
69
- found in outer plasma side portion of lipid bilayer | - anchors for 2 surface proteins: DAF (CD55) and MIRL (CD59
Phosphatidylinositol (PI)
70
DAF and MIRL float on surface of the membrane as they link to PI through a ____ with multiple sugars
Glycan core
71
Links CD55 and CD59 to PI
Phosphatidylinositol glycan Class A (PIG-A)
72
PIG-A mutation, ⬇️CD55 and CD59, | Cell susceptible to complement-mediated hemolyisis
PNH
73
Numerical naming of 15 proteins | Migration thru gel with velocity MW net charge
SDS PAGE
74
Dye to stain BANDS in sds page
Coomassie blue
75
Dye to stain GLYCOPHORINS (abundant barc side chain)
Periodic acid schiff dye (PAS dye)
76
Links band 3 to protein 4.1
Adducin
77
Links glut-1 to protein 4.1
Dematin
78
Principal skeletal proteins
Alpha and beta spectrins
79
Alpha and beta spectrins assempble to form ____ held by lateral bonds
Antiparallel heterodimer
80
COOH end ➖➖➖ NH3 end of other
Ntiparallel
81
Provides lateral membrane stability
Skeletal proteins / peripheral proteins (spectrins in hexagonal lattice)
82
Secondary structure of both alpha/beta spectrin features
Triple helical repeats of 106 amino acids each
83
20 triple helical repeats make up____
Alpha spectrin
84
16 triple helical repeats make up____
Beta spectrin
85
14-16 monomers whose length is regulated by TROPOMYOSIN
Actin
86
Cap the ends of ACTIN
Adducin | Tropomodulin
87
Stabilizes actin (?)
Dematin
88
Triple helix held by
Actin | 4.1
89
⏬vertical anchorages, Lipid membrane peels of in small blebs called
Vesicles
90
Autosomal dominant mutations affecting SPECTRIN DIMER TO DIMER LATERAL BONDS or SPECTRIN-ANKYRIN-4.1 junction
Hereditary elliptocytosis or OVALOCYTOSIS
91
Membrane fails to rebound from deformation ➡️ RBCs elongate to form _____ ➡️ mild to severe hemolytic ANEMIA
ELLIPTOCYTES
92
Rbc membrane permeable to:
H20 HCO3' Cl'
93
Regulate conc of Na and K
ATPase- dependent cation pumps
94
IC:EC ratio
Na+ 1:12 K+ 25:1
95
Pump mechanism damage permits the influx of ____. Cell swells ➡️ spheroid ➡️ ruptures ➡️ Hb spilled called ____
NA+ and H20 COLLOID OSMOTIC HEMOLYSIS
96
Extrudes calcium | IC: 5-10 umol/L
Ca2+ ATPase
97
A cytoplasmic Ca2+ binding protein, CONTROLS THE FUNCTION OF Ca2+ ATPase
Calmodulin
98
Increased Cation permeability | Sickle Hb deforms cell ➡️ HIGH IC Na, K, Ca ➡️ hemolysis
Sickle cell disease

Hema Lec 1 (1 decks)

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