RBC Metabolism Flashcards

1
Q

Identify the three areas of cel metabolism that are crucial for normal erythrocyte survival and function.

A

1) RBC membrane
2) Hemoglobin structure and funciona
3) RBC metabolic pathways

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2
Q

Is Glycophorin integral or peripheral? What is its function?

A

Integral, negative charge and location of antigens

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3
Q

Is Spectrin integral or peripheral? What is its function?

A

Peripheral, strengthen and perserve deformability

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4
Q

State the structural defects to produce Acanthocytes.

A

Increase in surface areas = Decrease in intracellular HGB

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5
Q

State the structural defects to produce Bite Cells

A

Decrease in Spectrin

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6
Q

State the structural defects to produce Sphereocytes

A

Decrease in Spectrin

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7
Q

State the structural defects to produce Target Cells

A

Increase in surface area = Decrease in intracellular HGB

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8
Q

What is the protein that delivers iron?

A

Transferrin

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9
Q

What are the two major tissues where heme synthesis occurs?

A

BM and liver

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10
Q

Draw Diagram of Heme synthesis! Begin with Succinyl Coenzyme A and glycine and end with heme

A

Back of PP

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11
Q

Describe the chemical structure of heme

A

2 Alpha Chains and 2 non alpha chains

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12
Q

What is the reason for “ringed sideroblasts?”

A

Mitochondrial Buildup of Iron

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13
Q

What is the reason for freshly voided urine from a patient with porphyria to not be red?

A

Has not oxidized yet

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14
Q

What are the three hemoglobins found in the embryo?

A

Grower 1, grower 2 and Portland

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15
Q

What is the composition and names of the 3 global chains and what are the percentages of each?

A
A = 2 alpha and 2 Beta, >95%
A2 = 2 Alpha and 2 Delta, 1.5-3%
F = 2 Alpha and 2 Gamma,
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16
Q

Characterize the oxygen affinity of R (relaxed) and T (tense) hemoglobin.

A

R is affinity for O2

T has a lower affinity for O2

17
Q

What happens in a left shift and where does it occur?

A

Uptake of O2 , lung

18
Q

What happens in a right shift and where does it occur?

A

Release of O2, Tissue

19
Q

What happens to PH, 2-3 DPG levels, and Temperature in a shift to the left?

A

PH increases
2,3 DPG levels decreases
Temperature decreases

20
Q

What happens to PH, 2-3 DPG levels, and Temperature in a shift to the right?

A

PH decreases
2,3 DPG levels increases
Temperature increases

21
Q

List the three abnormal hemoglobins that are unable to transport or deliver oxygen.

A

Carboxyhemoglobin
Methemoglobin
Sulfahemoglobin

22
Q

State the main source of ATP production in mature RBCs

A

Mitochondria

23
Q

Name that metabolic pathway that generates most of the RBC ATP.

A

Emden meyerhof Pathway

24
Q

State the major function of Eden Meyerhof pathway.

A

Anaerobic glycolysis = energy

25
Q

State the major function of Hexose monophosphate shunt.

A

Protects RBCs from environmental oxidants

26
Q

State the major function of Methemoglobin Reductase Pathway.

A

Maintains iron in the ferrous (2+) state

27
Q

State the major function of Leubering-Rapport Shunt.

A

Synthesis of 3,3 DPG

28
Q

State that changes in RCS leading to demise in 120 days

A

As enzymes break down, lose of energy production and deformability and can’t not get through he cords of the spleen = phagocytize by RES cells.

29
Q

Compare and contrast steps in extravascular Versus Intravascular breakdown of RBCs

A

(on back of objectives)
Extravascular = 90%, within RES cells, RES phagocytize, Iron is couples to transferrin, Bilirubin to Urobilinogen and excreted
Intravascular= 10%, within blood vessels, Haptoglobin pricks up free HGB, go to liver where Bilirubin to Urobilinogen and excreted

30
Q

What is the level of Haptoglobin in the presence of intravascular hemolysis?

A

Decreased

31
Q

State the main protein carrier for Bilirubin

A

Albumin

32
Q

State the main protein carrier for Hemoglobin

A

Haptoglobin

33
Q

State the main protein carrier for Iron

A

transferrin

34
Q

Define Porphyria

A

Disorder of heme synthesis

35
Q

Define Porphyrin

A

Oxidized porphyrinogen, stable, florescent pinkish-red “port wine”

36
Q

Define Porphyrinogen

A

Precursor to Porphyrin, colorless, non fluorescent, highly unstable. Quickly oxidizes to porphyrin