BCL10 Full Name
B-cell CLL/lymphoma 10
BCL10 Introduction
B-cell CLL/lymphoma 10 (BCL10) is a cytosolic adaptor protein that plays a pivotal role in the adaptive immune response by linking antigen receptor signaling to the activation of the NF-κB transcription factor. Structurally, BCL10 is characterized by an N-terminal Caspase Recruitment Domain (CARD) and a C-terminal serine/threonine-rich domain. Its primary physiological function is to act as a core component of the "CBM complex," which consists of CARD11 (also known as CARMA1), BCL10, and MALT1. Upon stimulation of T-cell (TCR) or B-cell receptors (BCR), BCL10 is recruited to the membrane, where it undergoes oligomerization to form filamentous structures. These BCL10 filaments serve as a platform to recruit MALT1, thereby facilitating the ubiquitination of downstream mediators like NEMO. This cascade ultimately results in the nuclear translocation of NF-κB, driving the expression of genes necessary for lymphocyte proliferation, survival, and cytokine production.
Figure 1. BCL10 structure and interactors. (Source: Gehring T, et al. 2018)
The clinical importance of BCL10 was originally identified through its involvement in Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma. In a significant subset of these lymphomas, a specific chromosomal translocation, t(1;14)(p22;q32), places the BCL10 gene under the control of the immunoglobulin heavy chain enhancer. This genetic aberration leads to the constitutive overexpression of BCL10, causing uncontrolled NF-κB activation that inhibits apoptosis and drives tumorigenesis. Beyond malignancy, BCL10 is critical for immune competency; rare germline mutations that result in BCL10 deficiency lead to a form of Combined Immunodeficiency (CID). These patients exhibit defective T and B cell responses, highlighting the protein's indispensable role in normal immune function. Conversely, somatic mutations in BCL10 have also been implicated in other diffuse large B-cell lymphomas (DLBCL), emphasizing its status as a frequent target in hematological malignancies.
Alternate Names for BCL10
BCL10
B-cell CLL/lymphoma 10
B-cell lymphoma/leukemia 10
c E10
CARD containing molecule enhancing NF kB
CARD containing apoptotic signaling protein
CARD containing proapoptotic protein
CARD like apoptotic protein
CARMEN
caspase recruiting domain containing protein
Cell Signaling
