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Flashcards in Haemoglobin Synthesis Deck (47):
1

Where does Haemoglobin Synthesis begin

Mitochondria

2

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

Glycine and succinyl CoA

3

Further reactions take place in the cytoplasm to form

Protoporphyrin IX

4

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

Ferrochelatase

5

This combines with ______ to form a haemoglobin molecule

Globlin chain

6

How many amino acids make up the alpha chain

141

7

How many amino acids make up the beta chain

146

8

What chains make up the normal adult Hb A

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

9

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

Alpha 2 delta 2
Alpha 2 Gamma 2

10

What is the least abundant haemoglobin molecule

HbF

11

What is the name of haemoglobin with O2

Oxyhemoglobin

12

What is the name of haemoglobin without O2

Deoxyhaemoglobin

13

What is the name of oxidized haemoglobin

Methemoglobin

14

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

Carboxyhemoglobin

15

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

Sulfhemoglobin

16

HbA1c is the name of what type of haemoglobin

Glycosylated Hemoglobin

17

What is the term used to describe a heme production problem

Porphyria

18

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

Hemochromatosis

19

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

Sickle cell disease, thalassemia

20

What is the name of an acquired disorder of heme synthesis

Lead poisoning

21

What is the name of a congenital disorder of heme synthesis

Porphyria

22

Lead poisoning inhibits what two compounds in heme production

Ferrochelatase and amino laevulinic acid dehydratase activities

23

Lead displaces what metal at enzyme active sites

Zn

24

What are some symptoms of lead poisoning

Mental confusion, severe abdominal pain in adults
Developmental and health problems in children

25

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

Porphyria

26

What is the origin of porphyria

Liver or erythropoietin

27

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

True

28

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

False, it’s 50 %

29

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

Porphyrins

30

Attacks due to Porphyria can be triggered by

Some chemicals, some food and exposure to sun

31

Treatment due to Polypuria attacks include administration of ________

Hemin

32

How does Hemin treat Polypuria

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

33

What are the two types of globin chain synthesis

Qualitative , Quantitative

34

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

Substitution of one amino acid molecule
Sickle cell gene

35

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

Defective rate of globin chain synthesis
Eg thalassaemia

36

Haemoglobin D is more prominent in what geographical location

Punjab region, India

37

Haemoglobin E is more prominent in what geographical location

South East Asia

38

Haemoglobin C is more prominent in what geographical location

African and Mediterranean

39

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

Valine, 6th, bets chains

40

What haemoglobin is a Structural variant of haemoglobin A

Haemoglobin C

41

What amino acid substitution occurs In Haemoglobin C

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

42

Try it False, HbA is less soluble than HbC

False Hb C is less soluble

43

What structures are formed in haemoglobin C

Rhomboidal crystals

44

What genotype will produce a milder Anaemia in Haemoglobin C patients

Homozygous state

45

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

HbS, HbA

46

What is the substitution mutation for Haemoglobin E

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

47

What type of haemoglobin is the second most common

HbE