Structure of RBCs Flashcards

1
Q

What is the Bombay phenotype?

A

A person that has an O blood type, regardless of the alleles present at the I loci. This is due to h/h or O/h genotype that prevents mutation of an intermediate needed to generate A or B antigens

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2
Q

RBCs do not adhere to vessel walls or other RBCs. How is this possible?

A

Due to the presence of glycophorin on the outer membrane, which gives RBCs a negative charge

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3
Q

The pentose phosphate pathway is a shunt of glycolysis. How is this pathway utilized by the RBC?

A

The PPP generates NADPH, which the RBC needs to maintain stores of reduced glutathione. Glutathione is used to detoxify ROS and prevent oxidation of hemoglobin to metehemoglobin.

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4
Q

This protein is present on young RBCs to prevent phagocytosis, but is lost with aging.

A

CD47

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5
Q

Rh (+/-) is determined by the presence/absence of what antigen?

A

D antigen - contributes to membrane stability

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6
Q

True/False. Scramblases require ATP for activation.

A

False. Flipases & flopases require ATP. Scramblases require Ca2+/

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7
Q

This pathway is a shunt of glycolysis that accounts for over 65% of an RBC’s ability to reduce methemoglobin to hemoglobin.

A

Methemoglobin Reductase/ Cytochrome b5 Reductase

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8
Q

Glycophorin is sometimes referred to as a decoy receptor. What does this mean?

A

The protein serves as a receptor for some pathogens; however, the pathogen cannot infect or replicate inside the RBC. The pathogen is essentially stuck and the infected RBC is removed by the spleen.

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9
Q

The H gene is responsible for the H antigen, which is present on all RBCs. The I gene has three potential alleles. What are they?

A

A allele - N-aceytlgalactosamine transferase

B allele - galactose transferase

O allele - frameshift mutation with no functional protein

These alleles and their encoded proteins are responsible for blood type.

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10
Q

What is the role of alpha and beta spectrin?

A

Cytoskeleton proteins that form antiparallel dimers that provide horizontal stability to the RBC and contribute to deformability

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11
Q

Bone marrow is primarily responsible for erythropoiesis in adults. Where does erythropoiesis take place in the developing fetus?

A

Yolk sac, spleen, liver

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12
Q

Phospholipids of the RBC membrane are distributed asymmetrically. What is the effect of the loss of this asymmetry?

A

Loss of asymmetry indicates an aging or abnormal RBC. These RBCs are detected by the spleen and destroyed.

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13
Q

True/False. RBCs have a biconcave disk shape that provides a large area-to-volume ratio for gas exchange and deformability.

A

True - enhances gas exchange and survival under various pressures

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14
Q

Glucose enters RBCs through the insulin-independent GLUT1. How do RBCs generate ATP?

A

Anaerobic glycolysis

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15
Q

This RBC defect is caused by a spectrin mutation that causes loss of horizontal stability and the progressive elongation of RBCs.

A

Hereditary Elliptocytosis

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16
Q

What is paroxysmal nocturnal hemoglobinuria?

A

A type of inherited hemolytic anemia due to the absence of CD55 & CD59, which serve to prevent complement activation against RBCs

17
Q

Methemoglobinuria is caused by a buildup of methemoglobin and is characterized by “chocolate blood” and cyanosis. How is this disorder treated?

A

Methylene blue - increases activity of the NADPH Methemoglobin Reductase

18
Q

What two complexes are responsible for anchoring the RBC membrane to the cytoskeleton?

A

Ankyrin complex, actin junctional complex

19
Q

What is the process of RBC aging on the membrane?

A

As the RBC ages, less ATP is produced and Ca2+ accumulates. This causes inhibition of the flipases and flopases and increased activation of scramblases. This ultimately causes loss of membrane asymmetry, which is recognized by macrophages in the spleen.

20
Q

What is a reticulocyte?

A

The final immature form of an RBC that is released by bone marrow. They lack many organelles and a nucleus and mature in the blood to become a RBC.

21
Q

What hormone stimulates RBC maturation and from where is this hormone release?

A

Erythropoietin released by the kidney

22
Q

This RBC defect is caused by a mutation to a protein in the ankyrin complex that causes loss of vertical stability.

A

Hereditary Spherocytosis - mutations to Band3, ankyrin, or spectrin that cause RBCs to become spherocytes without deformability

23
Q

The Rapoport-Leubering Pathway is a shunt of glycolysis. What is the product of this pathway and its effect on oxygen delivery to cells?

A

Produces 2,3-BPG, which reduces hemoglobin’s affinity for O2 and promotes O2 delivery to tissues

24
Q

These proteins bind to the GPI anchor on RBCs to prevent complement activation.

A

CD55 & CD59