BCL10 immune signaling adaptor | Elisa - Clia - Antibody - Protein

Background

BCL10 (B-cell CLL/lymphoma 10) is a protein involved in immune signaling pathways, particularly those related to lymphocyte activation and the adaptive immune response. Initially identified due to its translocation in mucosa-associated lymphoid tissue (MALT) lymphomas, BCL10 is crucial for the proper functioning of the immune system. It plays a significant role in activating the NF-κB (nuclear factor kappa-light-chain-enhancer of activated B cells) signaling pathway, which is essential for immune responses, inflammation, and cell survival. Dysregulation of BCL10 activity is linked to various pathological conditions, including immunodeficiency, autoimmune diseases, and lymphomas.

Protein Structure

BCL10 is composed of the following structural features:

• CARD Domain (Caspase Recruitment Domain): Located at the N-terminus, this domain mediates protein-protein interactions, essential for the formation of signaling complexes.
• Serine/Threonine-Rich Region: Contains multiple phosphorylation sites that are critical for its regulation and function.
• Interaction Motifs: These motifs allow BCL10 to bind to other signaling molecules, facilitating the assembly of signal transduction complexes.

Classification and Subtypes

BCL10 belongs to the family of CARD-containing proteins, which are involved in various signaling pathways, particularly those related to apoptosis and inflammation. It does not have distinct subtypes but interacts with several other proteins in signaling pathways, such as MALT1 (Mucosa-associated lymphoid tissue lymphoma translocation protein 1) and CARMA1 (CARD-containing MAGUK protein 1).

Function and Biological Significance

BCL10 plays a pivotal role in the following:

• NF-κB Activation: It is essential for the activation of the NF-κB signaling pathway in lymphocytes. Upon antigen receptor engagement, BCL10 forms a complex with MALT1 and CARMA1, leading to the activation of IKK (IκB kinase) and subsequent NF-κB activation.
• Immune Response Regulation: By activating NF-κB, BCL10 regulates the expression of various genes involved in immune responses, inflammation, and cell survival.
• T and B Cell Function: It is critical for the proper functioning of both T and B lymphocytes, influencing their proliferation, differentiation, and survival.

Clinical Issues

Abnormalities in BCL10 function or expression are associated with several clinical conditions:

• MALT Lymphoma: Translocations involving the BCL10 gene are frequently observed in MALT lymphomas, leading to constitutive NF-κB activation and uncontrolled cell proliferation.
• Immunodeficiencies: Mutations in BCL10 can lead to primary immunodeficiencies, characterized by impaired immune responses and increased susceptibility to infections.
• Autoimmune Diseases: Dysregulation of BCL10 activity can contribute to the development of autoimmune conditions by affecting the balance of immune regulation.

Summary

BCL10 is a crucial immune signaling adaptor protein that mediates the activation of the NF-κB pathway, essential for immune responses, inflammation, and cell survival. It plays a vital role in the function of T and B lymphocytes and is implicated in various clinical conditions, including lymphomas, immunodeficiencies, and autoimmune diseases. Understanding the mechanisms underlying BCL10's function and regulation offers potential therapeutic targets for treating related disorders.