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The thymus (pl.: thymuses or thymi) is a specialized primary lymphoid organ of the immune system.Within the thymus, thymus cell lymphocytes or T cells mature. T cells are critical to the adaptive immune system, where the body adapts to specific foreign invaders.
The remaining cells exit the thymus as mature naive T cells, also known as recent thymic emigrants. [13] This process is an important component of central tolerance and serves to prevent the formation of self-reactive T cells that are capable of inducing autoimmune diseases in the host.
Congenital athymia's clinical symptoms are directly related to the thymus's absence and its incapacity to generate T cells with the necessary immune capabilities. An increased vulnerability to bacterial, viral, and fungal infections results from T-cell immunodeficiency. [1] These patients have an especially high incidence of pneumonias.
T cell deficiency is a deficiency of T cells, caused by decreased function of individual T cells, it causes an immunodeficiency of cell-mediated immunity. [1] T cells normal function is to help with the human body's immunity, they are one of the two primary types of lymphocytes (the other being B cells ).
AIRE is a transcription factor expressed in the medulla [broken anchor] (inner part) of the thymus. It is part of the mechanism which eliminates self-reactive T cells that would cause autoimmune disease. It exposes T cells to normal, healthy proteins from all parts of the body, and T cells that react to those proteins are destroyed.
Nezelof syndrome is an autosomal recessive [6] congenital immunodeficiency condition due to underdevelopment of the thymus.The defect is a type of purine nucleoside phosphorylase deficiency with inactive phosphorylase, this results in an accumulation of deoxy-GTP which inhibits ribonucleotide reductase.
The symptoms are very similar to graft-versus-host disease (GVHD). This is because the patients have some T cells with limited levels of recombination with the mutant RAG genes. These T cells are abnormal and have a very specific affinity for self antigens found in the thymus and in the periphery.
A thymocyte is an immune cell present in the thymus, before it undergoes transformation into a T cell. [1] Thymocytes are produced as stem cells in the bone marrow and reach the thymus via the blood.