Sandbox Reserved 1455: Difference between revisions
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== Disease == | == Disease == | ||
Mutated RAG-1 or RAG-2 prevents a fully functional immune system from developing. Defects in RAG1 or RAG2 cause impaired V(D)J recombination and this leads to defective expression of the pre-TCR and pre-BCR, a critical event in the development of T cells and B cells. In vivo experiments with RAG1 or RAG2 deficiency reveal that the complex can be partially mutated. In this case, the semifunctional RAG complex is linked to Omenn Syndrome. Omenn Syndrome is associated with severe immunodeficiency. | |||
== Relevance == | == Relevance == | ||