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Hemoglobin Dr. Sarita Patel ASSISTANT PROFESSOR BIOCHEMISTRY, GMCS NORMAL ADULT HUMAN HEMOGLOBIN Structure and Function of Hb Oxygen dissociation curve P50 ??? T & R form of Hb Structure and Function of Hb Co-operative binding of oxygen Bohr effect Bohr’s effect Role of 2,3 BPG • 2,3 BPG level • Increase – High altitude – Chronic lung disease – Anemia • Decrease- Hypophosphatemia 2,3 BPG has less affinity for HbF • Gamma globin and βglobin proteins are 72% identical at the amino acid level • one significant amino acid difference exists in the 2,3BPG binding pocket. • In the γglobin protein, His 143 is replaced by a Ser in the 2,3BPGbinding site. • The effect of this amino acid substitution is the loss of two positive charges (one from each γ subunit relative to the β subunits). • 2,3 BPG IN STORED BLOOD???? • Biochemical changes in stored whole blood – PH? – K+? – 2,3 BPG – Plateletes • Role of Preservative for storage of whole blood? CARBAMINO-HEMOGLOBIN Hb combine with C02. • Methemoglobin • Carboxyhemoglobin • Regulation in erythroid cell. – Regulated by Ferochelatase and Uroporphyrinogen Synthase – No Rate limiting step. • Reason of Pain and neuropsychiatric symptoms???? • Reason of Photosensitivity? • Anemia is not a predominant feature Precipitating Factor • • • • • • Drinking alcohol Excessive intake of iron Smoking Fasting Drugs like Barbiturates, Sedatives Sun exposure Treatment • Symptomatic treatment for – Pain – Nausea, Vomitting – Intravenous Hemin – Intravenous Glucose – Avoidance of triggering factor – Antioxidant – vitamin E, beta carotene. Role of Glucose in Porphyria • ALA synthase enzyme is a rate limiting enzyme. • Transcription of ALA synthase is upregulated by Peroxisome Proliferator activated receptor coactivator-1 (PGC-1). • PGC-1 is regulated by Glucose level. • Hypoglycemia induce PGC-1 production Increase ALA synthase Increase Heme production Normal red cell breakdown haemoglobin haem iron transferrin globin protoporphyrin CO Expired air Amino acids Bilirubin (free) Liver conjugation erythroblast Bilirubin glucuronides Urobilin(ogen) Urine Stercobilin(ogen) faeces
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