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Basic Haemotology (1204) > Haemoglobin Synthesis > Flashcards

Haemoglobin Synthesis Flashcards

1
Q

Where does Haemoglobin Synthesis begin

A

Mitochondria

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2
Q

The condensation of what two compounds in the mitochondria produces amino laevulinic acid (ALA)

A

Glycine and succinyl CoA

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3
Q

Further reactions take place in the cytoplasm to form

A

Protoporphyrin IX

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4
Q

What enzyme catalysts the insertion of ferrous iron into protoporphyrin IX to form heme

A

Ferrochelatase

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5
Q

This combines with ______ to form a haemoglobin molecule

A

Globlin chain

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6
Q

How many amino acids make up the alpha chain

A

141

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7
Q

How many amino acids make up the beta chain

A

146

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8
Q

What chains make up the normal adult Hb A

A

2 alpha globin chains
2 beta globin chains
Gene: porphyrin plus Fe2+

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9
Q

What is the make up of the Minors Hb in
HbA2
Hb F

A

Alpha 2 delta 2

Alpha 2 Gamma 2

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10
Q

What is the least abundant haemoglobin molecule

A

HbF

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11
Q

What is the name of haemoglobin with O2

A

Oxyhemoglobin

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12
Q

What is the name of haemoglobin without O2

A

Deoxyhaemoglobin

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13
Q

What is the name of oxidized haemoglobin

A

Methemoglobin

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14
Q

What is the name of the haemoglobin with 218 times affinity to CO than O2

A

Carboxyhemoglobin

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15
Q

What is the name of the haemoglobin in re to sulfur drugs

A

Sulfhemoglobin

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16
Q

HbA1c is the name of what type of haemoglobin

A

Glycosylated Hemoglobin

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17
Q

What is the term used to describe a heme production problem

A

Porphyria

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18
Q

What is the name of the disease used when an individual has Fe2+ problems

A

Hemochromatosis

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19
Q

What is the name of the disease associated with a Globin problem

A

Sickle cell disease, thalassemia

20
Q

What is the name of an acquired disorder of heme synthesis

A

Lead poisoning

21
Q

What is the name of a congenital disorder of heme synthesis

A

Porphyria

22
Q

Lead poisoning inhibits what two compounds in heme production

A

Ferrochelatase and amino laevulinic acid dehydratase activities

23
Q

Lead displaces what metal at enzyme active sites

A

Zn

24
Q

What are some symptoms of lead poisoning

A

Mental confusion, severe abdominal pain in adults

Developmental and health problems in children

25
Q

What is name of the disease associated with a deficiency of enzymes in heme synthesis

A

Porphyria

26
Q

What is the origin of porphyria

A

Liver or erythropoietin

27
Q

True or False, majority of Porphyrias are inherited in an autosomal order

A

True

28
Q

True or False, less than 30% of individuals have normal levels of enzymes that can synthesize some heme

A

False, it’s 50 %

29
Q

Affected individuals have an accumulation of _________, which can be toxic at high concentrations

A

Porphyrins

30
Q

Attacks due to Porphyria can be triggered by

A

Some chemicals, some food and exposure to sun

31
Q

Treatment due to Polypuria attacks include administration of ________

A

Hemin

32
Q

How does Hemin treat Polypuria

A

Negative feedback for Heme Biosynthetic pathway thereby preventing accumulation of heme precursors

33
Q

What are the two types of globin chain synthesis

A

Qualitative , Quantitative

34
Q

What is the meaning of quantitative disorder of globin chain synthesis and give examples

A

Substitution of one amino acid molecule

Sickle cell gene

35
Q

What is the meaning of a Quantitative disorder of globin chain synthesis and give an example

A

Defective rate of globin chain synthesis

Eg thalassaemia

36
Q

Haemoglobin D is more prominent in what geographical location

A

Punjab region, India

37
Q

Haemoglobin E is more prominent in what geographical location

A

South East Asia

38
Q

Haemoglobin C is more prominent in what geographical location

A

African and Mediterranean

39
Q

What is the name of the amino acid that replaces Glutamic Acid on the ______ position of both _______ chains

A

Valine, 6th, bets chains

40
Q

What haemoglobin is a Structural variant of haemoglobin A

A

Haemoglobin C

41
Q

What amino acid substitution occurs In Haemoglobin C

A

Glutamic Acid replaces lysine st the 6th position in beta haemoglobin chain

42
Q

Try it False, HbA is less soluble than HbC

A

False Hb C is less soluble

43
Q

What structures are formed in haemoglobin C

A

Rhomboidal crystals

44
Q

What genotype will produce a milder Anaemia in Haemoglobin C patients

A

Homozygous state

45
Q

In Haemoglobin D, electrophoretic mobility is similar to ________ in alkaline solution, and similar to ________ in acidic solution

A

HbS, HbA

46
Q

What is the substitution mutation for Haemoglobin E

A

Lysine for glutamic Acid at the 26th position of the beta haemoglobin chain

47
Q

What type of haemoglobin is the second most common

A

HbE

Basic Haemotology (1204) (16 decks)

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