Haemoglobin Synthesis

Question 1

Where does Haemoglobin Synthesis begin

Answer 1

Mitochondria

Question 2

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

Answer 2

Glycine and succinyl CoA

Question 3

Further reactions take place in the cytoplasm to form

Answer 3

Protoporphyrin IX

Question 4

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

Answer 4

Ferrochelatase

Question 5

This combines with ______ to form a haemoglobin molecule

Answer 5

Globlin chain

Question 6

How many amino acids make up the alpha chain

Answer 6

141

Question 7

How many amino acids make up the beta chain

Answer 7

146

Question 8

What chains make up the normal adult Hb A

Answer 8

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

Question 9

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

Answer 9

Alpha 2 delta 2

Alpha 2 Gamma 2

Question 10

What is the least abundant haemoglobin molecule

Answer 10

HbF

Question 11

What is the name of haemoglobin with O2

Answer 11

Oxyhemoglobin

Question 12

What is the name of haemoglobin without O2

Answer 12

Deoxyhaemoglobin

Question 13

What is the name of oxidized haemoglobin

Answer 13

Methemoglobin

Question 14

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

Answer 14

Carboxyhemoglobin

Question 15

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

Answer 15

Sulfhemoglobin

Question 16

HbA1c is the name of what type of haemoglobin

Answer 16

Glycosylated Hemoglobin

Question 17

What is the term used to describe a heme production problem

Answer 17

Porphyria

Question 18

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

Answer 18

Hemochromatosis

Question 19

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

Answer 19

Sickle cell disease, thalassemia

Question 20

What is the name of an acquired disorder of heme synthesis

Answer 20

Lead poisoning

Question 21

What is the name of a congenital disorder of heme synthesis

Answer 21

Porphyria

Question 22

Lead poisoning inhibits what two compounds in heme production

Answer 22

Ferrochelatase and amino laevulinic acid dehydratase activities

Question 23

Lead displaces what metal at enzyme active sites

Answer 23

Zn

Question 24

What are some symptoms of lead poisoning

Answer 24

Mental confusion, severe abdominal pain in adults

Developmental and health problems in children

Question 25

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

Answer 25

Porphyria

Question 26

What is the origin of porphyria

Answer 26

Liver or erythropoietin

Question 27

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

Answer 27

True

Question 28

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

Answer 28

False, it’s 50 %

Question 29

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

Answer 29

Porphyrins

Question 30

Attacks due to Porphyria can be triggered by

Answer 30

Some chemicals, some food and exposure to sun

Question 31

Treatment due to Polypuria attacks include administration of ________

Answer 31

Hemin

Question 32

How does Hemin treat Polypuria

Answer 32

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

Question 33

What are the two types of globin chain synthesis

Answer 33

Qualitative , Quantitative

Question 34

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

Answer 34

Substitution of one amino acid molecule | Sickle cell gene

Question 35

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

Answer 35

Defective rate of globin chain synthesis | Eg thalassaemia

Question 36

Haemoglobin D is more prominent in what geographical location

Answer 36

Punjab region, India

Question 37

Haemoglobin E is more prominent in what geographical location

Answer 37

South East Asia

Question 38

Haemoglobin C is more prominent in what geographical location

Answer 38

African and Mediterranean

Question 39

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

Answer 39

Valine, 6th, bets chains

Question 40

What haemoglobin is a Structural variant of haemoglobin A

Answer 40

Haemoglobin C

Question 41

What amino acid substitution occurs In Haemoglobin C

Answer 41

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

Question 42

Try it False, HbA is less soluble than HbC

Answer 42

False Hb C is less soluble

Question 43

What structures are formed in haemoglobin C

Answer 43

Rhomboidal crystals

Question 44

What genotype will produce a milder Anaemia in Haemoglobin C patients

Answer 44

Homozygous state

Question 45

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

Answer 45

HbS, HbA

Question 46

What is the substitution mutation for Haemoglobin E

Answer 46

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

Question 47

What type of haemoglobin is the second most common

Answer 47

HbE