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Hemoglobin & Derivatives: DR. S. Irfan Raza

Here are the key points from the lecture: - Normal hemoglobin types include HbA, HbA2, HbF - Abnormal hemoglobins include HbS, HbC, HbE, HbD - HbS is the most common abnormal hemoglobin - HbE is common in parts of Pakistan, India, Sri Lanka and Bangladesh - Abnormal hemoglobins can be identified through electrophoresis - HbS provides resistance to malaria because it has a shorter lifespan and causes sickling, which lyses the red blood cells - Thalassemias are genetic blood disorders caused by reduced synthesis of the globin chains of hemoglobin

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Syed Irfan Raza
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183 views

Hemoglobin & Derivatives: DR. S. Irfan Raza

Here are the key points from the lecture: - Normal hemoglobin types include HbA, HbA2, HbF - Abnormal hemoglobins include HbS, HbC, HbE, HbD - HbS is the most common abnormal hemoglobin - HbE is common in parts of Pakistan, India, Sri Lanka and Bangladesh - Abnormal hemoglobins can be identified through electrophoresis - HbS provides resistance to malaria because it has a shorter lifespan and causes sickling, which lyses the red blood cells - Thalassemias are genetic blood disorders caused by reduced synthesis of the globin chains of hemoglobin

Uploaded by

Syed Irfan Raza
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PDF, TXT or read online on Scribd
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Hemoglobin Introduction

Structure of Hemoglobin
Functions of Hemoglobin
Heme containing Compounds

Hemoglobin & Derivatives


DR. S. Irfan Raza
Hemoglobin (Hb)
 Hemoglobin is found exclusively in RBCs

 Its main function is to transports O2 and CO2 between lungs


and various tissues.

 Normal concentration of Hb-A (Major Hemoglobin) in Adults


 Adult males: 135 – 175 g/L 13.8 to 17.2 (g/dL)
 Adult females: 120 – 168 g/L 12.1 to 15.1 (g/dL)
Hemoglobin Structure & Functions
It is an oxygen/CO2 carrier protein present in the RBCs of blood. It is a
conjugated chromo-protein having heme as its prosthetic group.
Heme is the prosthetic group, not only of hemoglobin but also of myoglobin,
cytochromes etc.
Hemoglobin Functions

Its performing two important functions:

1. Delivery of Oxygen
2. Transport of CO2
Hemoglobin Structure
Properties of iron in heme
Fe forming how many coordination complexes?

Coordination number of
iron in heme = 6

6 bonds:
• 4x pyrrole ring (A,B,C,D)
• 1x link to a protein
• 1x link to an oxygen
Quaternary Structure of Hemoglobin
•Hb comprises of:
•1. dimer of (αβ)1 2. dimer of (αβ)2

•Each dimer is held together by:


•Many Hydrophobic interactions.
Compounds Containing Heme Group

Hb

Peroxidases
Mb
Heme
containing
Compounds

Catalases Cyto
Hemoglobin Derivatives

Hb

CO Met
Hb HB
Methemoglobin
When hemoglobing has Fe3+ state i.e., Methhemoglobin loses its
ability to release oxygen. Patient suffers with methemoglobinmia
in which Hemoglobin cant release oxygen to tissues effectively.
Methemoglobin Causes

H2O2

Free
Bromates Methhemoglobin Radicals

Chlorayes
CO is a toxic compound and has 200 times higher affinity than O2 for Binding with Hb.
Carboxyemoglobinemia

Headache

Irritability Nausea

CO
Hemoglobin

Breathless
Vomiting ness
Abnormal Hemogobins

Sickle Cell
anemia

Hemoglobino
pathies

Thelasse
Porphyria's
mias
Viva Questions:
Define hemoglobin and its structure

What Methemoglobin and related clinical conditions

What is Carboxyhemoglobin and its causes and related


clinical conditions

Enlist Hemoglobinopathies.

How body converts methemoglobin back to


hemoglobin.

Thank You.
Lecture - 2 Hemoglobin Related Diseases

Dr. Syed Irfan Raza


 Abnormal Hemoglobin

 Sickle Cell Anemia

 Occurrence

 Molecular Basis – Homozygous & Heterozygous states

 Sickle Cell Related Abnormalities

 Mechanism of Sickling

 Sticky Patches
 Types of Normal Hb

 Types of Abnormal Hb

 Sickle Cell trait provides resistance to malaria

 HbS diagnosis

 Management

 Hemoglobin c disease

 Hemoglobin D

 Hemoglobin E
Normal Major Types of Hb

HbA1
90%

HbA1c
Normal HbA2
Hb 5%

HbF2%
Sickle Cell Trait Provides Resistance To Malaria

 HbS Shorter life span

 HbS has low pH cause sickling of HbS cell which results into
lysis

 Low Intra HbS K+ level


Sickling Test:
Microscopic examination of blood (Na-dithionite)
Diagnosis
 Electrophoresis - @ pH8.6 HbS moves slowly towards anode
– HbS less negative.
Management
1. Use of Sodium Cyanate
Na-cyanate inhibit sickling of erythrocytes.

Cyanate increases the affinity of O2 to HbS &


lowers the formation of deoxy HbS – side effect of peripheral
nervous system

Patients with severe anemia needs repeated blood transfusion


which result into iron over load leads to cirrhosis of liver.
Abnormal Hemoglobin Types

HbS

HbE Hb HbC

HbD
Hemoglobin C Disease
Hemoglobin C disease is a condition affecting a protein in the blood
(hemoglobin) which transports oxygen throughout the body.
Symptoms of this condition can include fatigue, weakness,
and anemia .
The spleen can also become enlarged as a result of this disease.

Cooleys hemoglobinemia (HbC)

Genetic Alteration
Glu – Lys @ 6th position of B chain.

Occurrence: Only in blacks

Characterized by mild hemolytic anemia.

Therapy Availble: No therapy


Hemoglobin E:
Hemoglobin E (HbE) is an abnormal hemoglobin with a single
point mutation in the β chain.

Most common abnormal hemoglobin after HbS.

Occurrence:
10% of south east Asia population.
Indian, Sri Lankan and Bangladeshi, few areas of Pakistan

Genetic Mutation:
Glu – Lys @ 6th AA – B-chain.

Clinical Importance:
No clinical manifestation.
Hemoglobin D:
Genetic Mutation:
121 codon (GAA→CAA)
Glu – Gln @ 121st AA – B-chain.

There can be several variants from different places


(HbD – Punjab), (HbD – LosAngles)

Diagnosis-
On electrophoresis moves along with HbS.
 Types of Normal Hemoglobin

 Enlist abnormal hemoglobin

 Which type of Abnormal hemoglobin is


most common

 Which type of hemoglobin is common in


Pakistan

 How Abnormal hemoglobin can be


identified

 How HbS provides resistance to malaria

 What is thalassemias?

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