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Sickling Erythrocytes: An Indian Perspective

Neeru Dhamija and Anita Garg Mangla

Since 2009, 19th June every year is marked as World Sickle cell day. As per World health organization (WHO), nearly 5% of world’s population has haemoglobin disorders. Hemoglobin (Hb) is a heterotetramer with two alpha and two beta subunits. Major contributors of Hb disorders are sickle cell disease (SCD) and Thalessemia. HBB gene (Cytogenetic Location: 11p15.4) that codes for Hb beta subunit produces low levels of beta globin when mutated. This condition is known as beta thalessemia (1) while alpha thalessemia is a result of mutation in alpha subunit gene decreasing its protein levels. SCD is a group of disorders resulting from mutations of Hb beta subunit. One such mutation is a single base substitution, A to T, that causes sickle cell anaemia (SCA) which is most common SCD


 
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