Structure and

Structure and
function of
function of
Haemoglobin
Haemoglobin
Dr. Tariq M Roshan
Dr. Tariq M Roshan
Department of Hematology
Department of Hematology
PPSP
PPSP

The main function of red blood cell

The main function of red blood cell

Transfer of O Transfer of O2 2 from lungs to tissue from lungs to tissue

Transfer of O Transfer of O2 2 from tissue to lungs from tissue to lungs

To accomplish this function red cells

To accomplish this function red cells
has haemoglobin !Hb"
has haemoglobin !Hb"

#ach red cell has $%& million

#ach red cell has $%& million
molecules of Hb
molecules of Hb
'ntroduction
'ntroduction

'ntroduction
'ntroduction

Haemoglobin !Hb"( protein constituting )*+

Haemoglobin !Hb"( protein constituting )*+
of the red blood cells
of the red blood cells

Synthesis begins in proerythroblast

Synthesis begins in proerythroblast

$,- at erythroblast stage $,- at erythroblast stage

+,- at reticulocyte stage +,- at reticulocyte stage

T.o parts
T.o parts

Haem Haem

/lobin /lobin

Synthesis of Haemoglobin
Synthesis of Haemoglobin
!Hb"
!Hb"

Haem 0 globin produced at t.o

Haem 0 globin produced at t.o
di1erent sites in the cells
di1erent sites in the cells

Haem in mitochondria Haem in mitochondria

/lobin in polyribosomes /lobin in polyribosomes

2ell synchroni3ed
2ell synchroni3ed

Synthesis of Haemoglobin
Synthesis of Haemoglobin

Synthesis of Haem
Synthesis of Haem

Protoporphyrin ring .ith an iron atom

Protoporphyrin ring .ith an iron atom
in centre
in centre

The main site is mitochondria as it

The main site is mitochondria as it
contains 454S
contains 454S

Mature red cell does not contain

Mature red cell does not contain
mitochondria
mitochondria

Structure of Haem
Structure of Haem

Synthesis of globin
Synthesis of globin

Synthesis of globin
Synthesis of globin

6arious types of globin combines .ith

6arious types of globin combines .ith
haem to from di1erent haemoglobin
haem to from di1erent haemoglobin

#ight functional globin chains(

#ight functional globin chains(
arranged in t.o clusters the
arranged in t.o clusters the

7 cluster ! 7 cluster !

( (

( (

and and

globin genes" on the globin genes" on the

short arm of chromosome )) short arm of chromosome ))

7 cluster ! 7 cluster !

and and

globin genes" on the short globin genes" on the short

arm of chromosome )$ arm of chromosome )$

/lobin gene clusters
/lobin gene clusters

/lobin synthesis( starts at +
/lobin synthesis( starts at +
rd rd
.ee8 of
.ee8 of
gestation
gestation

#mbryonic
#mbryonic


Haemoglobin /o.er ' !
Haemoglobin /o.er ' !

2 2

2 2
"
"


Haemoglobin Portland !
Haemoglobin Portland !

2 2

2 2
"
"


Haemoglobin /o.er '' !
Haemoglobin /o.er '' !

2 2
2
2
"
"

9etal : Hb9 !
9etal : Hb9 !

2 2

2 2
"( Hb4 !
"( Hb4 !

2 2

2 2
"
"

4dult : Hb4( Hb42 !

4dult : Hb4( Hb42 !

2 2

2 2
"( Hb9.
"( Hb9.
Synthesis of globin
Synthesis of globin

/lobin chain s.itch
/lobin chain s.itch

4lpha 0 beta chains
4lpha 0 beta chains

Hb 4
Hb 4
Hb 4
Hb 4
2 2
Hb 9
Hb 9
structure
structure

2 2

2 2

2 2

2 2

2 2

2 2
;ormal -
;ormal -
<$7<= -
<$7<= -
).,7+.2 -
).,7+.2 -
&.,7&.= -
&.,7&.= -
4dult haemoblobin
4dult haemoblobin

9unctions of Haemoglobin
9unctions of Haemoglobin

O>ygen deli?ery to the tissues

O>ygen deli?ery to the tissues

Reaction of Hb 0 o>ygen
Reaction of Hb 0 o>ygen

O>ygenation not o>idation O>ygenation not o>idation

One Hb can bind to four O One Hb can bind to four O2 2 molecules molecules

5ess than .&) sec required for o>ygenation 5ess than .&) sec required for o>ygenation

chain mo?e closer .hen o>ygenated chain mo?e closer .hen o>ygenated

2hen o>ygenated 2(+7DP/ is pushed out 2hen o>ygenated 2(+7DP/ is pushed out

chains are pulled apart .hen O chains are pulled apart .hen O2 2 is is
unloaded( permitting entry of 2(+7DP/ unloaded( permitting entry of 2(+7DP/
resulting in lo.er a@nity of O resulting in lo.er a@nity of O2 2

O>y 0 deo>yhaemoglobin
O>y 0 deo>yhaemoglobin

O>ygen7haemoglobin
O>ygen7haemoglobin
dissociation cur?e
dissociation cur?e

O
O2 2
carrying capacity of Hb at
carrying capacity of Hb at
di1erent Po
di1erent Po2 2

Sigmoid shape
Sigmoid shape

Ainding of one molecule facilitate the second Ainding of one molecule facilitate the second
molecule binding molecule binding

P P ,& ,& !partial pressure of O !partial pressure of O2 2 at .hich Hb is at .hich Hb is

half saturated .ith O half saturated .ith O2 2" 2$.$mmHg " 2$.$mmHg

Hb7o>ygen dissociation
Hb7o>ygen dissociation
cur?e
cur?e

The normal position of cur?e

The normal position of cur?e
depends on
depends on

oncentration of 2(+7DP/ oncentration of 2(+7DP/

H H
B B
ion concentration !pH" ion concentration !pH"

O O
2 2
in red blood cells in red blood cells

Structure of Hb Structure of Hb
Hb7o>ygen dissociation
Hb7o>ygen dissociation
cur?e
cur?e

Right shift !easy o>ygen deli?ery"

Right shift !easy o>ygen deli?ery"

High 2(+7DP/ High 2(+7DP/

High H High H
B B

High O High O
2 2

HbS HbS

5eft shift !gi?e up o>ygen less readily"

5eft shift !gi?e up o>ygen less readily"

5o. 2(+7DP/ 5o. 2(+7DP/

Hb9 Hb9
Hb7o>ygen dissociation
Hb7o>ygen dissociation
cur?e
cur?e

Summary
Summary

;ormal structure including the proportion of

;ormal structure including the proportion of
globin chains are necessary for the normal
globin chains are necessary for the normal
function of haemoglobin
function of haemoglobin

Reduced haemoglobin in the red blood cells

Reduced haemoglobin in the red blood cells
due to any abnormality of any of its
due to any abnormality of any of its
constituents result into a clinical situation
constituents result into a clinical situation
called anaemia
called anaemia

Metabolic 0 other abnormalities result into

Metabolic 0 other abnormalities result into
abnormal o>ygen supply to the tissue
abnormal o>ygen supply to the tissue