What do RAG1 and RAG2 do?
The RAG1 and RAG2 proteins initiate V(D)J recombination by introducing double-strand breaks at the border between a recombination signal sequence (RSS) and a coding segment.
What is the function of RAG protein?
The recombination-activating genes (RAGs) encode parts of a protein complex that plays important roles in the rearrangement and recombination of the genes encoding immunoglobulin and T cell receptor molecules.
Where is RAG1 found?
RAG1 and RAG2 are, respectively, located at chromosome positions 11p12 and 11p13 and encode for the RAG1 and RAG2 proteins. RAG proteins are lymphoid-specific components of the complex of enzymes initiating the V(D)J recombination process (Fugmann et al., 2000).
Where are RAG genes expressed?
Recombination activating genes (RAG)1 and RAG2 are expressed in developing B and T lymphocytes and are required for the rearrangement of antigen receptor genes. In turn, RAG expression is regulated by the products of these assembled immunoglobulin (Ig) and T-cell receptor (TCR) genes.
What are RAG1 mice?
RAG-1-deficient mice have small lymphoid organs that do not contain mature B and T lymphocytes. The arrest of B and T cell differentiation occurs at an early stage and correlates with the inability to perform V(D)J recombination. The immune system of the RAG-1 mutant mice can be described as that of nonleaky scid mice.
What is RAG in B cells?
RAGE-ligands interaction induces a series of signal transduction cascades and lead to the activation of transcription factor NF- B as well as increased expression of cytokines, chemokines, and adhesion molecules.
What is RAG gene?
Recombination-activating gene 1 (RAG1) and RAG2 (referred to collectively here as RAG genes) encode lymphoid-specific proteins that are expressed during the early stages of T cell and B cell development and initiate the process of V(D)J recombination by introducing DNA double-strand breaks (DSBs) at the junction …
What is RAG expression?
What is RAG deficiency?
Altered T and B cell development in patients with RAG gene defects is associated with impairment of immunological tolerance. Ultimately, the degree in the severity of T and B cell lymphopenia, breakage of immune tolerance, and exposure to environmental triggers are the main factors that shape the clinical phenotype.
Which chain of the TCR involves Vdj?
β-chain
D-to-J recombination occurs first in the β-chain of the TCR.