HEMOGLOBIN

Question 1

found in the rbc of the body

Answer 1

hemoglobin

Question 2

each rbc contains approximately ____ hemoglobin molecules

Answer 2

280 million hemoglobin molecules

Question 3

main function of rbc

Answer 3

transport of oxygen from the lungs to the body’s cell

Question 4

rbc contains a protein called

Answer 4

hemoglobin

Question 5

carries oxygen

Answer 5

hemoglobin

Question 6

hemoglobin molecule is composed of a protein group, known as

Answer 6

globin

Question 7

hemoglobin have __ heme groups, associated with an iron atom.

Answer 7

4 heme groups

Question 8

1 heme = ___ of oxygen and 1 hemoglobin molecule = ____ of oxygen

Answer 8

1 mole of oxygen ; 4 moles of oxygen

Question 9

in synthesis of hb molecule, the heme portion begins..

Answer 9

Heme Portion: begins in the mitochondria with the formation of D-ALA from glycine and succinyl coenzyme A

Question 10

in synthesis of hb molecule, the globin portion begins..

Answer 10

Globin portion: produced on specific ribosome in the cytoplasm of the red blood cells

Question 11

The globin in each hemoglobin molecule consists of ___ polypeptide chains which
determine the type of hemoglobin formed.

Answer 11

four

Question 12

Gene involved for Alpha & Zeta chains:

Answer 12

chromosome 16

Question 13

Gene involved for Beta, Delta, Epsilon & Gamma chains:

Answer 13

chromosome 11

Question 14

hb in Adult

Answer 14

HbA1 > 95%
HbA2 1.5-3%
HbF <2%

Question 15

hb in neonate

Answer 15

-HbF 60-80%
-HbA 20-40%

Question 16

This is the designation for the normal hemoglobin that exists after birth. Hemoglobin __ is a tetramer with two alpha chains and two beta chains (a2b2).

Answer 16

Hemoglobin A

Question 17

This is a minor component of the hemoglobin found in red cells after birth and consists of two alpha chains and two delta chains (a2d2).

Answer 17

Hemoglobin A2

Question 18

Hemoglobin F is the predominant hemoglobin during fetal development. The molecule is a tetramer of two alpha chains and two
gamma chains (a2g2).

Answer 18

Hemoglobin F.

Question 19

This the predominant hemoglobin in people with sickle cell disease. The alpha chain is normal.

Answer 19

Hemoglobin S

Question 20

in hemoglobin S, Valine replaces

Answer 20

GLUTAMIC ACID at 6th position

Question 21

results from a mutation in the beta globin
gene and is the predominant hemoglobin found in people with hemoglobin __ disease (a2bC2).

Answer 21

Hemoglobin C.

Question 22

relatively benign, producing a mild hemolytic
anemia and splenomegaly.

Answer 22

Hemoglobin C.

Question 23

in hemoglobin c , lysine replaces

Answer 23

GLUTAMIC ACID at 6th position

Question 24

People with this disease have a mild hemolytic anemia and mild splenomegaly. this trait is benign and is extremely common in S.E. Asia and in some areas equals hemoglobin A in frequency.

Answer 24

Hemoglobin E

Question 25

in Hemoglobin E, lysine replaces

Answer 25

GLUTAMIC ACID at 26th position

Question 26

a variant in which a mutation in the alpha globin gene produces an alpha globin chain that is
abnormally long.

Answer 26

Hemoglobin Constant Spring.

Question 27

The quantity of hemoglobin in the cells for hemoglobin CS is low for two reasons.

Answer 27

First, the messenger
RNA for hemoglobin Constant Spring is unstable. Some is degraded prior to
protein synthesis. Second, the Constant Spring alpha chain protein is itself unstable.

Question 28

result of hemoglobin constant spring

Answer 28

The result is a thalassemic phenotype. From family of ethnic Chinese background
from the Constant Spring district of Jamaica

Question 29

a tetramer composed of four beta globin
chains and forms in people with three-gene alpha thalassemia as well as in people with
the combination of two-gene deletion alpha thalassemia and hemoglobin
Constant Spring.

Answer 29

Hemoglobin H

Question 30

Hemoglobin Barts develops in fetuses with four-gene deletion alpha thalassemia.

During normal embryonic development, the episilon gene of the alpha globin gene locus
combines with genes from the beta globin locus to form functional hemoglobin molecules.

The episilon gene turns off at about 12 weeks, and normally the alpha gene takes over. With
four-gene deletion alpha thalassemia no alpha chain is produced.

Answer 30

Hemoglobin Barts

Question 31

hemoglobin barts was named

Answer 31

From St. Bartholomew’s Hospital in London. The hospital has the fond sobriquet,
St. Barts, and the hemoglobin was named “hemoglobin Barts.”

Question 32

Hemoglobin is made of two subunits derived from genes in the alpha gene
cluster on chromosome 16 and two subunits derived from genes in the beta
gene cluster on chromosome 11.

Answer 32

Compound Heterozygous Conditions

Question 33

Having two different variant genes from the alpha globin gene cluster or two
different variant genes from the beta globin gene cluster (a gene for
hemoglobin S and one for hemoglobin C)

Answer 33

Compound Heterozygous Conditions

Question 34

Patients with hemoglobin SC disease inherit a gene for hemoglobin S from one parent, and a gene for hemoglobin C from the other. Hemoglobin C interacts with hemoglobin S to produce some of the abnormalities seen in patients with sickle cell disease. On average, patients with hemoglobin ____disease have milder symptoms than do those with sickle cell disease.

Answer 34

Hemoglobin SC disease

Question 35

In this condition, the patient has inherited a gene for hemoglobin S from one parent and a gene for beta-thalassemia from the other. Has large quantity of normal hemoglobin produced by the beta-thalassemia gene. (Thalassemia genes produce normal hemoglobin, but in variably reduced amounts).

Answer 35

Sickle/beta-thalassemia

Question 36

The combination of hemoglobin E and beta-thalassemia produces a
condition more severe than is seen with either hemoglobin E trait or
beta-thalassemia trait.

moderately severe thalassemia (thalassemia intermedia)

most common in people of S.E. Asian background.

Answer 36

Hemoglobin E/beta-thalassemia

Question 37

Has alpha globin gene cluster

The alpha globin gene cluster on one of the two chromosomes 16 has
both alpha globin genes deleted. On the other chromosome 16, the
alpha1 gene has the Constant Spring mutation.

produces a severe shortage of alpha globin chains.

The excess beta chains associate into tetramers to form hemoglobin H.

Answer 37

Alpha thalassemia/Hemoglobin Constant Spring.