• Dr. Awadhesh Kumar Baranwal
• Dr. Sujit Kumar

HbS Sickle Cell Anemia is a homozygous type of Sickle Cell Anemia (HbSS). A single point substitution of valine for glutamine 6 in the -globin chain causes this. This decreases red blood cell solubility, resulting in polymerization and vascular obstruction. The -globin gene is found on the short arm of chromosome 11. When two -globin mutant subunits are joined, haemoglobin S is produced (HbS). In low-oxygen circumstances, the absence of a polar amino acid at the six-point position of the -globin chain promotes non-covalent haemoglobin polymerization, altering the composition and decreasing the flexibility of sickle red blood cells. When normal oxygen voltage is restored, these cells do not return to their original state. As a result, as these compact blood cells travel through small capillaries, they are unable to deform, resulting in artery obstruction and Ischemia. The underlying illness anaemia is caused by hemolysis, or the breakdown of red cells within the spleen.

Sickle cell anemia, Genetics disorder, pathophysiology etc.

"A Literary Study on Sickle Cell Anaemia (HbS): A Gene Defect Disorder ", International Journal of Emerging Technologies and Innovative Research (www.jetir.org), ISSN:2349-5162, Vol.8, Issue 9, page no.e500-e509, September-2021, Available :http://www.jetir.org/papers/JETIR2109460.pdf

"A Literary Study on Sickle Cell Anaemia (HbS): A Gene Defect Disorder ", International Journal of Emerging Technologies and Innovative Research (www.jetir.org | UGC and issn Approved), ISSN:2349-5162, Vol.8, Issue 9, page no. ppe500-e509, September-2021, Available at : http://www.jetir.org/papers/JETIR2109460.pdf