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
2
Q
the essential enzyme in HMP
A
glucose-6-phosphate dehydrogenase
3
Q
end products of EMP (2)
A
- pyruvate
- lactate
4
Q
T/F:
EMP does not generate atp
A
False
5
Q
In EMP, what is the enzyme deficiency?
clue: isipin mo product ng EMP
A
Pyruvate Kinase Deficiency
6
Q
Pyruvate Kinase Deficiency would produce what type of red cell?
A
Echinocyte/crenated/Burr cell
same lang yan
7
Q
Give a function of RBC that does not need ATP
A
Oxygen Transport
8
Q
how many atp consumed in glycolysis?
A
2 ATP
9
Q
Ferrous or Ferric
which forms methemoglobin?
A
Ferric Iron
10
Q
characteristics of ferric iron
A
- forms methemoglobin/hemiglobin
- can’t bind O2; makes hemoglobin dysfunctional
- reversible
11
Q
sodium-potassium ATPase pump forms what rbc?
A
stomatocytes
12
Q
RBC with mouth-like appearance central pallor
A
stomatocytes
13
Q
PATHWAY
detoxifies peroxide (H2O2)
A
HMP Shunt
14
Q
enzyme deficiency in HMP shunt
A
G6PD deficiency
15
Q
G6PD deficiency will cause.. (3)
A
- denatured Hemoglobin (Heinz bodies)
- supravital stain (RHH)
- bite cells
16
Q
HMP Shunt
what can be seen with supravital stain
RHH
A
- reticulocytes
- Heinz bodies
- Hemoglobin H
17
Q
products of HMP shunt
A
- reduced glutathione (GSSG)
- NADPH
18
Q
MRP is also called..
A
Cytochrome B5 Reductase
19
Q
Methemoglobin Reductase Pathway can convert _ back to _
A
ferric back to ferrous
20
Q
enzyme deficiency in MRP
A
cytochrome b5 deficiency
21
Q
If you are deficient in Cytochrome B5, your blood color is..
A
chocolate brown
22
Q
pathway
generates 2,3-diphosphoglycerate
A
Rapoport-Luebering Pathway
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
24
Q
high or low
if 2,3-BPG is high, delivery of O2 to tissues is _
A
high
25
RBC membrane is composed of: (3)
* protein (50%)
* lipid (40%)
* CHO (10%)
26
# RBC membrane
predominate in the **outer** layer (2)
* phosphatidylcholine
* sphingomyelin
27
# RBC membrane
predominate in the **inner** layer (2)
* phosphatidylserine
* phosphatidylethanolemine
28
_ and _ constitute the principal RBC membrane structure
cholesterol and phospholipids
29
_ are associated with abnormalities in the conc or distribution of membrane cholesterol and phospholipids
acanthocytosis & target cells
30
other name for acquired acanthocytosis
Spur cell anemia
31
this is an alcoholic chronic liver disease
acquired acanthocytosis (spur cell anemia)
32
# Transmembrane Proteins
2 major macromolecular complexes
* ankyrin complex
* actin junctional complex
33
provide support and to connect the lipid bilayer to the underlying cytoskeleton to maintain membrane integrity
Transmembrane proteins
34
# Transmembrane proteins
water transporter
Aquaporin 1
35
# Transmembrane proteins
Anion transporter
Band 3
36
# which is not a transmembrane protein?
* Band 3
* Glycophorin B
* Ankyrin
* ICAM-4
Ankyrin
37
# Transmembrane or Skeletal Protein?
Protein 4.1
Skele
38
# Transmembrane or Skeletal Protein?
RhAG
TM
39
# Transmembrane or Skeletal Protein?
Duffy
TM
40
# Transmembrane or Skeletal Protein?
Glut-1
TM
41
# Transmembrane or Skeletal Protein?
Kell
TM
42
# Transmembrane or Skeletal Protein?
a-spectrin
skele
43
# Transmembrane or Skeletal Protein?
adducin
skele
44
# Transmembrane or Skeletal Protein?
G3PD
skele
45
# Skeletal Protein
anchors the actin junctional complex to spectrin tetramers
Protein 4.1
46
# Skeletal protein
filamentous antiparallel heterodimer
a-spectrin
47
# Transmembrane Proteins
_ and _ support the majority of the ABH system
* Band 3
* Glut-1
48
# Transmembrane Proteins
carries the peptide-defined **M and N** determinants
Glycophorin A
49
# Transmembrane Proteins
carried the **Ss **determinants
Glycophorin B
50
# Transmembrane Proteins
_ and _ together comprise the MNS system
* Glycophorin A
* Glycophorin B
51
# Transmembrane Proteins
Glycophorin C and D carry the..
Gerbich System Antigens
52
RH null results in _
stomatocytes/stomatocytosis
53
PI serves as a base on which a glycan core of sugar molecule is synthesized, forming the
glycosylphosphatidylinositol
54
# Enumerate
GPI anchored proteins (2)
* Decay Accelerating Factor (CD55)
* Membrane Inhibitor of Reactive Lysis (CD59)
55
if there are no CD55 and CD59, the person is prone to _
complement mediated hemolysis
| aka Paroxysmal Nocturnal Hemoglobinuria
56
what is used to detect Paroxysmal Nocturnal Hemoglobinuria?
flow cytometry
57
An acquired mutation in this gene affects the cells' ability to synthesize the GPI anchor
## Footnote
without it, the cell memb becomes CD55 and CD59 deficient
Phosphatidylinositol Glycan Anchor Biosynthesis Class A** (PIGA)**
58
Principal cytoskeletal proteins (2)
* alpha-spectrin
* beta-spectrin
59
# cytoskeletal proteins
_ arises in the defects of **vertical** support
Hereditary spherocytosis
60
# cytoskeletal proteins
_ is due to defects in **horizontal** support
Hereditary elliptocytosis
61
_ synthesis occurs first and last in the _
heme synthesis; mitochondrion
62
enzyme that binds iron to protoporphyrin to form heme
**Ferrochelatase** (Fe 2+) aka Heme synthase
63
sensitive to light; burgundy urine
porphyria
64
how many heme groups are attached to four globin chains?
4 heme grps
65
what RBC stage are heme & globin components first synthesized?
pronormoblast
66
Protoporphyrin IX + Fe2+ = ...
Heme
67
what gives RBC its red color?
heme
68
Hemoglobinn is first present in the _
polychromatic normoblast
69
# Globin
2 identical pairs of unlike polypeptide chains
2 alpha, 2 non alpha
70
# chromosome
codes for Alpha and Zeta
Chromosome 16 (short arm)
71
# chromosome
codes for delta and gamma
Chromosome 11 (short arm)
## Footnote
beta, gama, delta, epsilon
72
# T/F:
alpha genes are 2 copies per chromatid
True
| so 4 alpha genes (2 from mom, 2 from dad)
73
globin chain of Gower 1
2 zeta + 2 epsilon
74
globin chain of Gower 2
2 alpha + 2 epsilon
75
globin chain of portland
2 zeta + 2 gamma
76
Hgb F is primary hgb until _ months
6 mos of age
77
what binds in the beta gene promoter region?
Kruppel-like Factor 1** (KLF-1)**
78
approximately _ mL of oxygen is bound by each gram of Hgb
1.34 mL
79
# Left or Right Shift
Low delivery of O2 to tissues
Left
80
# Left or Right Shift
reduced P50 / <27mmHg
Left
81
# Left or Right Shift
increased P50 / > 27mmHg
Right
82
# Left or Right Shift
alkalosis
Left
83
# Left or Right Shift
high body temp
Right
84
# Left or Right Shift
relaxed state
Left
85
# Left or Right Shift
acidosis
Right
| acidosis = low pH
86
a **shift** in the curve due to **change in PH**
Bohr Effect
87
# state
low affinity for oxygen
Tensed state
| shift to right
88
# state
deoxygenated
Tensed state
89
# state
High affinity for oxygen
Relaxed state
90
# Enumerate
dysfunctional hemoglobins (3)
* methemoglobinemia
* sulfhemoglobinemia
* carboxyhemoglobinemia
91
# enumerate
blood color of dysfunctional hemoglobin (3)
* methemoglobinemia: chocolate brown
* sulfhemoglobinemia: mauve lavender
* carboxyhemoglobinemia: cherry red
92
# dysf hemoglobin
which has **mauve lavender** blood?
sulfhemoglobinemia
93
# Enumerate
Methemoglobin (3 types)
* acquired/toxic methemoglobinemia
* M Hemoglobin
* Cytochrome B5 Reductase Deficiency
94
treatment for methemoglobin
methylene blue
| injected intravenously; reducing agent
95
# monoxide or dioxide?
carboxyhemoglobin is a combination of carbon _ with heme iron
monoxide
96
which dysfunctional hemoglobin is irreversible?
Sulfhemoglobin
97
color/pigment in sulfhemoglobin
greenish pigment
98
cyanmethemoglobin method measures all forms of Hgb except _
sulfhemoglobin
99
diagnosis of carbon monoxide poisoning in smorkers
> 10%
| if nonsmoker, > 3%
100
# dysfunctional hemoglobin
detected at 630 nm
* methemoglobin
* sulfhemoglobin
101
# dysfunctional hmoglobin
detected at 540 nm
carboxyhemoglobinemia
102
treatment for carboxyhemoglobinemia
hyperbaric oxygen therapy
