White Review Flashcards

1
Q

When oxygen binds to hemoglobin what happens to the iron atom

A

Causes a conformational change in which the iron atom moves into the plane of the heme ring (a 0.4 A change)

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2
Q

What does the movement of the iron cause within the Hb

A

the F8 proximal histidine to be pulled which changes the associated globin change

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3
Q

What must the binding of O2 be within the Hb

A

Reversible in order to allow the O2 to be attached in the lungs and detached at the tissue

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4
Q

How does O2 bind to Hb

A

In a cooperative fashion

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5
Q

T/F Conformational change in the heme ring following O2 causes conformational change in attached hemes making the affinity of O2 to the heme rings to increase

A

True

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6
Q

Is the pH of actively respiring tissues higher or lower then normal

A

Lower (more acidic)

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7
Q

What is the drop of pH in actively respiring tissue

A

0.2, 7.4 -> 7.2

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8
Q

What happens to the binding affinity of Hb for O2 as pH decreases

A

It also decreases

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9
Q

What happens in order to cause the release of the O2 from the Hb

A

A H+ is picked up from the tissue which causes a conformational change favoring the release of the O2

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10
Q

T/F Fetal Hb (HbF) needs to have a lower affinity to O2 then mother’s Hb

A

False, if it has a lower affinity O2 will not separate from maternal Hb causing fetal hypoxia

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11
Q

How does alpha globin chain affect Hb in fetal life

A

Excess gamma globin chains form gamma4 tetramers also known as Hb Bart’s

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12
Q

How does alpha globin chain affect Hb in adult life

A

Excess beta globin chains form beta4 tetramers also known as HbH

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13
Q

HbH disease affects how many genes

A

3 of the alpha genes are affected -a/–

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14
Q

What is HbH disease characterized by

A

Moderately severe anemia, Microcytic, hypochromic hemolytic anemia, hepatosplenomegaly, mild jaundice

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15
Q

What makes up Heinz bodies

A

HbH beta4 tetramers that precipitate slowly as red blood cells mature and age

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16
Q

What do Heinz bodies lead to

A

Hemolytic anemia

17
Q

What genes are affected with Hb Bart’s

A

Both alpha genes completely inactivated, –/–, leading to Hydrops fetalis

18
Q

What are the outcomes of Hydrops fetalis

A

Stillborn or death following right after birth, fetal onset of generalized edema and severe hypochromic anemia

19
Q

Are there any treatments for Hydrops fetalis

A

Few patients can be saved by exchange transfusions

20
Q

What are the pathological response to Hydrops Fetalis

A

Due to Hb Bart’s high affinity to O2 oxygen is bound to the HbF from maternal Hb however almost none is released to the fetal tissue leading to severe hypoxia