KIR3DL2

KIR3DL2 is a gene that encodes a member of the killer cell immunoglobulin-like receptor (KIR) family in humans. This family is a group of transmembrane glycoproteins expressed by natural killer cells and subsets of T cells. The KIR genes are polymorphic and highly homologous and they are found in a cluster on chromosome 19q13.4 within the 1 Mb leukocyte receptor complex (LRC).

Function

The KIR proteins are classified by the number of extracellular immunoglobulin domains (2D or 3D) and by whether they have a long (L) or short (S) cytoplasmic domain. KIR proteins with the long cytoplasmic domain transduce inhibitory signals upon ligand binding via a immune response process, while KIR proteins with the short cytoplasmic domain lack the inhibitory signal transduction apparatus and instead associate with the TYRO protein tyrosine kinase binding protein to transduce activating signals.

The KIR3DL2 protein is an inhibitory receptor that contributes to the fine tuning of the immune system. It is involved in the recognition of certain types of human leukocyte antigen (HLA) class I molecules.

Clinical significance

Alterations in KIR3DL2 expression have been associated with various diseases. For example, increased expression of KIR3DL2 has been observed in Sezary syndrome, a rare form of cutaneous T-cell lymphoma. This has led to the development of therapeutic strategies targeting KIR3DL2 in this disease.

See also

• Killer-cell immunoglobulin-like receptor
• Natural killer cell
• Sezary syndrome