What is the function of ZAP-70?

The ZAP70 gene provides instructions for making a protein called zeta-chain-associated protein kinase. This protein is part of a signaling pathway that directs the development of and turns on (activates) immune system cells called T cells. T cells identify foreign substances and defend the body against infection.

What is TCR signaling?

A TCR signal causes global cellular changes within the T cell ranging from the activation of transcriptional regulators and protein synthesis to the reorganization of the cytoskeleton and altered metabolism and are necessary for a naive T cell to undergo clonal expansion and differentiation into effector subsets.

What is zap70 deficiency?

ZAP-70 deficiency is a rare combined immunodeficiency (CID) with an autosomal-recessive pattern of inheritance. The disease was first described in 1989 in a patient of Mennonite descent with CD8 lymphocytopenia [1].

What does TCR mean in immunology?

T-cell receptor
T-cells are a subset of lymphocytes that play a large role in the immune response. The TCR (T-cell receptor) is a complex of integral membrane proteins that participate in the activation of T-cells in response to an antigen.

What is IgVH mutation?

IgVH mutation is a significant prognostic marker in chronic lymphocytic leukemia (CLL). IgVH mutation analysis combined with FISH, ZAP-70, and beta-2 microglobulin measurement provide comprehensive prognostic assessment and may be used to determine the approach to therapy for all CLL patients.

What is the prognosis of patients with ZAP-70 deficiency and what possible therapies are recommended?

The ZAP-70 deficiency is fatal unless treated by hematopoietic stem cell transplantation Hematopoietic Stem Cell Transplantation Hematopoietic stem cell (HSC) transplantation is a rapidly evolving technique that offers a potential cure for hematologic cancers (leukemias, lymphomas, myeloma) and other hematologic …

What is TCR in oncology?

T Cell Receptor Engineering (TCR) Like CAR T-cell therapy, engineered T cell receptor therapy involves treating cancer with activated T lymphocytes from the body. Both strategies attach new receptors to the cells’ surfaces, enabling them to attack different forms of cancer.

What does the TCR receptor do?

The T-cell receptor (TCR) is a protein complex found on the surface of T cells, or T lymphocytes, that is responsible for recognizing fragments of antigen as peptides bound to major histocompatibility complex (MHC) molecules.

Do IGHV mutated CLL patients progress?

IGHV status and prognosis from the time of diagnosis The 5-year OSd was 71% (95% CI: 68-74) for U-CLL patients and 81% (95% CI: 79-83) for those with M-CLL. The 5-year TFSd for U-CLL patients was 31% (95% CI: 27-35), compared with 68% (95% CI: 65-70) for those with M-CLL.

Is CD28 required for T cell activation?

CD28 is a major costimulatory receptor that is constitutively expressed on naive T cells and is essential for the activation of naive T cells by antigen recognition.