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India is a vast, ethnically diverse country and the people inhabiting it are as diverse as the land itself. Finally, it is planned to establish competent International collaborations for successfully achieving the objectives of the project and to develop competence and human resources in the area of SCA research. Patent search at all stages of project will be an integral part of the project. normal Hb, (b) white blood count, and (c) plasma gelsolin. (4) Development and on-ground implementation of an affordable, accurate and accelerated diagnostic kit which will provide simultaneous quantitative estimation of: (a) sickle/aggregated vs. The corrected hiPSCs will be reprogrammed to the blood lineage and can be an unlimited source for cell replacement therapy and it is proposed to validate functional efficacy of gene-corrected HSCs using ex vivo erythroid differentiation and engraftment into NSG mice. This approach will involve dedifferentiating the peripheral blood mononuclear cells of SCA patients to human induced pluripotent stem cells (hiPSCs), which will further undergo gene editing by using CRISPR-Cas9 to correct the SCA mutation. (3) Genome editing and stem cell research approach for the treatment of SCA. It is also proposed to explore the co-crystallization of drugs currently being prescribed for the management of SCA. This project will also facilitate the process of getting the DCGI approval for hydroxyurea therapy for SCA in India and conduct phase I/II clinical trials of a herbal formulation to be developed based on the plants (or their substitute plants in India) used in the preparation of Niprisan. This will involve screening pure natural/ synthetic/ semisynthetic compounds and botanicals against various validated sickle cell anaemia targets such as sickle shape modulation, induction of fetal γ-globin expression, Nitric Oxide inducers, inhibitors of Histone methyl/acetyl transferases, Phosphodiesterases, Cell-adhesion, HbS polymerization, NLRP3 inflammasome, inflammatory cytokines and pain management. (2) Discovery and development of new lead molecules for management of SCA to improve the quality of life with better life expectancy. With this consideration we propose a project with holistic approach for management of SCA which will include (1) population screening, genetic testing and counselling for SCA in selected districts of Chhattisgarh having high prevalence of this disease or trait for management of genetic burden of SCA as well as understanding the genetic basis of clinical course/differential response to hydroxyurea (HU) therapy. Hence early and affordable detection, treatment as well as preventive measures are important in managing this disease. The disease management is usually symptomatic and hydroxyurea is the major therapeutic agent that is available for its treatment. For example, although SCA does not confer resistance to infection, it protects from severe malaria but mortality in such patients is higher once malaria develops. A major cause of premature mortality in sickle disease patients is infection. The clinical course is also known to be variable and several factors including genetic constitution of an individual is said to be important. Their lifespan is usually reduced to 42-48 years with 50% of children with SCA dying before the age of 5. Individuals suffering from sickle cell disease show symptoms such as body pain, clotting, dyspnea, anaemia, jaundice, pneumonia, repeated infection etc. Due to irregular shape of these cells, they are prone to physical stress leading to hemolysis and capillary occlusion. The sickle shaped cells lose flexibility with reduced oxygen carrying capacity and induce dehydration in the cells. These fibers deform the disc shaped RBCs to sickle shaped cells.
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The mutated valine favours the hydrophobic interactions between the β subunits of the haemoglobin tetramers leading to the HbS polymerization and formation of long haemoglobin fibers. SCA is a genetic disease caused by a point mutation in the sixth codon of the β-globin expressing gene resulting in the replacement of glutamic acid by valine, which under deoxygenation state oligomerizes with α-globin and gives rise to a type of haemoglobin named as Haemoglobin sickle (HbS). Every year approximately 5,00,000 children are born with SCA worldwide with India accounting for nearly 50% of the cases. Sickle cell anaemia (SCA) is the most common blood related disorder in India with a high prevalence among ethnic groups that have a socio-economic disadvantage, such as tribal populations.