What is bare lymphocyte syndrome?

What is bare lymphocyte syndrome?

Bare lymphocyte syndrome (BLS) is an inherited condition of the immune system, and is known as a “primary immunodeficiency.” Children inherit the BLS gene from their parents. Both must carry the genetic mutation for BLS, but may not have symptoms of the disease.

What are the clinical manifestations of MHC class II deficiency?

Patients with MHC class II deficiency generally have severe CD4+ T-lymphocytopenia, hypogammaglobulinemia and lack of antigen-specific antibody responses. Proliferations to mitogen are usually conserved while absent to antigen.

What would happen to a person if they could not produce MHC proteins?

Without these, there would be no presentation of internal or external antigens to the T cells. The importance of MHC proteins is that they allow T cells to distinguish self from non-self. In every cell in your body, antigens are constantly broken up and presented to passing T cells.

What is the characteristic feature of TAP deficiency?

Although asymptomatic cases have been described, TAP deficiencies are usually characterized by chronic bacterial infections of the upper and lower airways, evolving to bronchiectasis, and in half of the cases, also skin ulcers with features of a chronic granulomatous inflammation.

What are the treatment options for patients with MHC class II deficiency?

Currently the only known cure for MHC class II deficiency is allogeneic hematopoietic cell transplantation (HCT).

Which adaptive immune responses would be affected by the MHC II deficiency?

Patients with MHC class II deficiency have a severe defect of cellular and humoral immune responses. CD4+ T cells are often decreased, while CD8+ T cells and B cells are present in normal numbers.

What is the difference between MHC I and MHC II?

MHC I molecules are expressed on all nucleated cells and are essential for presentation of normal “self” antigens. MHC II molecules are expressed only on the surface of antigen-presenting cells (macrophages, dendritic cells, and B cells). Antigen presentation with MHC II is essential for the activation of T cells.

What are the functions of MHC I and MHC II?

Major histocompatibility complex (MHC) class I and class II proteins play a pivotal role in the adaptive branch of the immune system. Both classes of proteins share the task of presenting peptides on the cell surface for recognition by T cells.

Which type of immune system cell is responsible for activating B cells?

helper T cells

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