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Dr. Niranjan Murthy H.

L
Assistant Professor
Dept of Physiology
SSMC

HAEMOGLOBIN
• INTRODUCTION
• STRUCTURE
• TYPES
• SYNTHESIS
• FATE
• DERIVATIVES
• CLINICAL- ANAEMIAS
- HAEMOGLOBINOPATHIES
- PORPHYRIAS

• Pigment present inside RBCs • Carries O2 and CO2 • Responsible for red color of blood • Deficiency is called anaemia .

polypeptide chain and a molecule of O2 .STRUCTURE OF HEMOGLOBIN • Iron-protoporhyrin-globin forms a subunit • 4 subunits join to form a molecule of Hb • Iron is in ferrous ( Fe2+) form • Fe2+ binds 4 pyrrole rings.

a • β and γ chain have 146 a.β.• 4 pyrroles join to form a ring called porphyrins • 4 polypeptide chains constitute globin • α.a .γ and δ are four important polypeptide chains • α chain has 141 a.

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Adult Type.α2β2 • HbA2.Fetal Type.α2δ2 • HbF.ζ2β2 .2% of adult Hb.ε2ζ2 • Hb gower2. TYPES OF HEMOGLOBIN • HbA.α2γ2 • Hb gower1.

SYNTHESIS SUCCINYL Co-A + GLYCINE ALA synthase δ-AMINOLEVUNIC ACID PORPHOBILINOGEN PROTOPORPHYRIN IX Fe2+ haem synthase HAEM GLOBIN HAEMOGLOBIN .

• Macrophages phagocytose – hemolyse - degrade haemoglobin – form Bilirubin – transport to liver with albumin – conjugated and detoxified – secreted in bile. . FATE OF HAEMOGLOBIN • RBCs are destroyed in RES ( mainly spleen and bone marrow) after 120 days of life.

H a e m o g lo b in H aem G lo b in P r o t o p o r p h y r in Iro n CO A m in o a c id p o o l B iliv e r d in ir o n p o o l B ilir u b in .

DERIVATIVES OF Hb 1) Oxyhaemoglobin (HbO2) – oxygenation reaction – combination with Fe 2+ 2) Carbaminohaemoglobin ( CO2Hb)  carbon dioxide combines with globin part 3) Carboxyhaemoglobin (COHb)  carbon monoxide binds with Fe 2+ { 250 times more affinity than oxygen} .

4) Methhaemoglobin ( HHb)  oxidized haemoglobin 5) Sulfhaemoglobin 6) Glycated haemoglobin (HbA1c)  Glucose attached to terminal Valine in β chain. – integrated index of Diabetic control over 4 to 6 weeks .

important in acid-base balance. Transports O2 from lungs to tissues in the form of oxy-hemoglobin • 2.6 times more than plasma proteins . Transports CO2 from tissues to lungs in the form of carbamino-hemoglobin:.30% of total CO2 transport • 3. Acts as a buffer.FUNCTIONS OF HEMOGLOBIN • 1.

APPLIED ASPECTS • Normal Hb value : Males  14 – 18 gm/dL Females  12 – 16 gm/dL # ANAEMIAS # HAEMOGLOBINOPATHIES # PORPHYRIAS .

< 5gm/dL . ANAEMIAS Classification: I . Based on severity. v) Mild – 8-12gm/dL vi) Moderate – 5-8gm/dL vii) Severe .

Acute blood loss • Macrocytic – eg Vit B12 & Folic acid deficiency • Microcytic hypochromic – iron deficiency anaemia .II) Based on morphology of RBCs • Normocytic normochromic – eg.

iron deficiency.thalassaemia . Surgeries • Haemolytic anaemias – congenital.III) Based on aetiology : • Blood loss – RTA. megaloblastic.Aplastic anaemia • Nutritional anaemias. • Genetic causes. malaria • Bone marrow depression.

Increased RBC sequestration . HAEMOGLOBINOPATHIES Sickle cell anaemia: Valine replaces glutamate in the 6th position of β chain. Is common in African blacks Confers resistance against malaria Hb crystallizes & takes sickle shape under hypoxic conditions.

Types:.Major & Minor β-thalassemia major cooley’s anaemia Treatment  # Transfusion – packed red cell is preferred. THALASSAEMIA • Reduced or absent synthesis of globin chains.alpha & Beta . # Splenectomy # Bone Marrow Transplant .

PORPHYRIAS • DEF: Inherited disorders involving specific enzymes in heme biosynthetic pathway • TYPES: • a) Hepatic porphyrias i) ALA dehydratase deficient porphyrias ii) Acute intermittent porphyrias iii) Porphyria cutanea tarda b) Erythropoetic porphyrias i) X-linked sideroblastic anemias ii) Congenital erythropoetic porphyrias .

combination with single polypeptide chain. ii) Neuroglobin – present in CNS iii) Cytochrome enzymes – present in mitochondria iv) Peroxidases .Other molecules containing Haem:- i) Myoglobin – present in muscles .