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Heme./Onc > Hemoglobin > Flashcards

Hemoglobin Flashcards

1
Q

What four subunits is HbA made of?

A

two alpha subunits

two beta subunits

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2
Q

What four subunits is HbA2 made of?

A

two alpha

two delta

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3
Q

What four subunits is HbF composed of?

A

two alpha

two gamma

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4
Q

What amino acid substitution is present in HbF? What can this new amino acid bind?

A

histidine to serine

23BPG

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5
Q

What chromosome are the alpha globins located?

A

16

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6
Q

What chromosome are the beta globins located?

A

11

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7
Q

HS-40 corresponds to what globin gene?

A

alpha

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8
Q

LCR corresponds to what globin chain?

A

beta

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9
Q

What is the amino acid substitution during HbS?

A

glutamate to valine

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10
Q

What drug is used to manage HbS? Why is this drug used?

A

Hydroxyurea

increases levels of HbF

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11
Q

What amino acid residue is present during HbC? What is thought to happen to HbC?

A

glutamic acid to lysine

crystallization

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12
Q

What amino acid substitution is present during HbS?

A

glutamate to valine

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13
Q

How many genes are there for α-globin? What chromosome?

A

four

16

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14
Q

In a person with severe (three gene deletion) α-thalassemia, what form of hemoglobin will form? What globin isotypes make this up?

A

Hb Barts

four gamma chains

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15
Q

When fetal Hb production winds down, what form of Hb will a person with severe α-thalassemia produce? What globin chain isotypes make this up?

A

HbH

four beta chains

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16
Q

Why are Hb Barts and HbH poor oxygen transporters?

A

too high of O2 affinity

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17
Q

What amino acid substitution is present during HbE? Where?

A

glutamate to lysine

southeast asia

18
Q

What is the only α-thalassemia that can be caused by a point mutation? What is produced?

A

Hb constant spring

excessively long α2 chain

19
Q

What type of recombination takes places in Hb Lepore?

A

fusion of beta and delta

20
Q

At a pH of 8.6, will HbA be positively or negatively charged?

A

negatively

21
Q

What amino acid of hemoglobin can protons bind? What other amino acid will this charged histidine now bind?

A

histidine

aspartate

22
Q

What is formed when CO2 binds Hb?

A

carbamates

23
Q

At what position is the amino acid substitution in HbS? What chain?

A

position 6

beta chain

24
Q

Which hemoglobinopathy has the same position and chain error as HbS?

A

HbC

25
Q

What position in the mutation in HbE? What chain? What happens to this globin?

A

26

beta chain

inefficient synthesis

26
Q

During HbLepore, what part of the mRNA contains delta globin? Beta globin?

A

delta = N-ter

beta = C-ter

27
Q

What is the function of HS-40 and LCR?

A

tissue specific expression of Hb

28
Q

What do HbS homozygotes have? Heterozygotes?

A

homozygotes = disease

heterozygotes = trait

29
Q

If four α-globin genes are deleted, what are the two isotypes of α-globin that can predominate?

A

Gower

Portland

30
Q

What is the error during Hb Constant Spring?

A

stop codon is changed to a glutamine residue

31
Q

Does Hb Constant Spring behave like a α+ or an α0 thalassemia?

A

α+

32
Q

What are the three Hb’s produced in the early embryo?

A

Gower 1, Gower 2 and Portland

33
Q

What does oxidized Fe3+ form?

A

hemichrome

34
Q

In what population is HbC confined?

A

West Africans

35
Q

What type of problems can accumulate in a patient with HbSC?

A

ocular

36
Q

Why can HbS and HbA be separated on an electrophoresis?

A

HbS is slightly more negative

37
Q

What restriction endonuclease is used for PCR HbS vs. HbA?

A

Mst II

38
Q

Other than polymerizing, what does the valine in HbS accomplish?

A

block O2 binding site on β-chain

39
Q

What is the amino acid substitution in Hb Helsinki? Where? What does this result in?

A

Lys to Met

2,3-BPG binding site

increased O2 affinity

40
Q

What is the amino acid substitution in Hb kansas? What does this result in?

A

Asn to Thr

decreased oxygen affinity

41
Q

What are the two hemoglobinopathies that readily form methemoglobin?

A

boston and hyde park

42
Q

What is the common error for almost all α-thalassemias? Due to what?

A

deletion

homologous recombination

Heme./Onc (15 decks)

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