TCR Gene Rearrangement and Organization

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What is the configuration of the TCR genes in the earliest cell entering the thymus?

Rearranged

What is the order of gene rearrangement if the γ chain genes rearrange successfully?

δ chain genes then β gene rearrangement

What is the role of recombination activation genes (RAGs) in T cells?

To activate the recombinase gene

What is the approximate number of different TCR structures that can be generated from αβ and γδ TCRs?

10^13

What is the process called when no further gene rearrangements occur after successful gene rearrangements?

Allelic exclusion

What happens if all the gene rearrangements are unsuccessful?

The second copy of genes on respective chromosomes will begin the rearrangement process

What is the function of RAG-1 and RAG-2 genes in the process of recombination?

To recognize specific DNA signal sequences

What is the difference between immuneoglobulin genes and genes encoding TCR?

Immunoglobulin genes do not undergo somatic mutation

Where do T lymphocytes develop?

In the thymus

What is the function of positive selection in the thymus?

To allow TCRs to recognize MHC molecules

What is the outcome of TCRs binding too strongly to p-MHC in the thymus?

Negative selection and elimination

What is the outcome of a defect in RAG-1 or RAG-2 genes?

Severe combined immunodeficiency (SCID)

What is the name of the cells that do not express either the CD4 or CD8 co-receptor?

Double-negative cells

At what stage do the cells decide to become δγ or αβ T cells?

At the double-negative stage

What percentage of thymocytes never become fully functional and die in the thymus?

96%-98%

What is the result of positive selection?

Down-regulation of RAG1 and RAG2 gene expression

What is the function of negative selection?

To remove T cells that express TCR with high reactivity to self components

What type of molecule is associated with the TCR β chain on the surface of pre-T cells?

Invariant pre-Tα molecule

What type of T-cells are MHC II restricted and recognize specific foreign peptides only when presented in association with specific MHC II molecules?

CD4 T-cells

Which type of T-cells produces cytokines of CMI and inflammation?

Th1 T-cells

What is the primary function of CD4 T-cells when recognizing antigen on the surface of APC?

Secreting cytokines

What is the term for the process by which T-cells that are unable to bind to MHC molecules are eliminated?

Negative selection

What is the primary function of CD8 T-cells?

Killing infected cells

What is the term for immature T-cells that express both CD4 and CD8?

Double-positive T-cells

What is the result of pre-TCR signaling in the development of cells expressing α and β as their TCR?

Further rearrangement of TCR β genes stops

What is the main function of positive selection in thymic selection?

Recognition of self-MHC and self-peptide

What is the composition of the pre-T cell receptor (pre-TCR)?

β chain and pre-Tα

What is the outcome of double positive cells that do not interact with MHC class I and II molecules?

They undergo apoptosis

What is the percentage of thymocytes that are double positive cells?

Over 80%

What is the role of thymic non-lymphoid cells in thymic selection?

Expressing MHC molecule plus peptide

Learn about the organization of human TCR genes and the order of TCR gene rearrangement, including the process of α and β chain genes rearrangement in the thymus and bone marrow. Understand how γ and δ chain genes rearrange and the implications on further gene rearrangement. Test your knowledge of TCR gene organization and rearrangement!

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