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In patients with Omenn Syndrome which gene (s) is/are usually deficient? Why does this affect both...

In patients with Omenn Syndrome which gene (s) is/are usually deficient? Why does this affect both B and T cells?

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During the Omenn Syndrome, there is mutation observed in genes in the immune system and the most frequent are due to mutations in RAG1 and RAG2 genes. The genes are responsible for instructing production of proteins which are active in B-cells and T-cells. The lymphocytes have special antigens on their surface that help them recognise foreign invaders and fight against it. Due to the mutation of the RAG1 and RAG2 genes it reduces production of the proteins active in B and T-cells and hence reduces its ability to recognise foreign invaders and fight against the infection.

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