# BCL10

Source: https://onco.cc/targets/bcl10/  
OnCo record `bcl10` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

BCL10 (B-cell lymphoma/leukaemia 10) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as an oncogene driver, a tumour suppressor, a biomarker and a fusion partner, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Non-Hodgkin lymphoma, Lung cancer, Diffuse large B-cell lymphoma and 1 more.

## Summary

Plays a key role in both adaptive and innate immune signalling by bridging CARD domain-containing proteins to immune activation. Acts by channeling adaptive and innate immune signalling downstream of CARD domain-containing proteins CARD9, CARD11 and CARD14 to activate NF-kappa-B and MAP kinase p38 (MAPK11, MAPK12, MAPK13 and/or MAPK14) pathways which stimulate expression of genes encoding pro-inflammatory cytokines and chemokines. Recruited by activated CARD domain-containing proteins: homooligomerised CARD domain-containing proteins form a nucleating helical template that recruits BCL10 via CARD-CARD interaction, thereby promoting polymerisation of BCL10, subsequent recruitment of MALT1 and formation of a CBM complex.

CIViC holds 1 clinical evidence item and 0 assertions across 1 variant. Open Targets scores its association with cancer at 0.69 (direct and indirect evidence; datatypes literature 0.80, genetic association 0.19, somatic mutation 0.88). IntOGen calls it a driver in 3 cohorts (1 activating, 2 loss-of-function), covering Diffuse Large B-Cell Lymphoma, NOS, Malignant Lymphoma.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: BCL10 immune signaling adaptor; B-cell lymphoma/leukemia 10; CARMEN; CIPER; mE10; c-E10
- Tags: cancer-genes-wave
- Symbol: BCL10
- Class: tumor-suppressor
- Biology: Plays a key role in both adaptive and innate immune signalling by bridging CARD domain-containing proteins to immune activation. Acts by channeling adaptive and innate immune signalling downstream of CARD domain-containing proteins CARD9, CARD11 and CARD14 to activate NF-kappa-B and MAP kinase p38 (MAPK11, MAPK12, MAPK13 and/or MAPK14) pathways which stimulate expression of genes encoding pro-inflammatory cytokines and chemokines. Recruited by activated CARD domain-containing proteins: homooligomerised CARD domain-containing proteins form a nucleating helical template that recruits BCL10 via CARD-CARD interaction, thereby promoting polymerisation of BCL10, subsequent recruitment of MALT1 and formation of a CBM complex. This leads to activation of NF-kappa-B and MAP kinase p38 (MAPK11, MAPK12, MAPK13 and/or MAPK14) pathways which stimulate expression of genes encoding pro-inflammatory cytokines and chemokines. Activated by CARD9 downstream of C-type lectin receptors; CARD9-mediated signals are essential for antifungal immunity. Activated by CARD11 downstream of T-cell receptor (TCR) and B-cell receptor (BCR). Location: Cytoplasm, perinuclear region; Membrane raft (UniProt). Locus 1p22.3 (HGNC).
- Where found: Non-Hodgkin lymphoma: Open Targets association 0.71 with non-Hodgkin lymphoma (MONDO_0018908); IntOGen driver in 1 cohort (MLYM); Lung cancer: Open Targets association 0.50 with lung cancer (MONDO_0008903); Diffuse large B-cell lymphoma: Open Targets association 0.54 with diffuse large B-cell lymphoma (MONDO_0018905); CIViC evidence names this disease; Marginal zone lymphoma: Open Targets association 0.58 with marginal zone lymphoma (MONDO_0017604)

## Notes

- Written by scripts/fetch-cancer-genes.ts from CIViC, Open Targets, IntOGen, HGNC and UniProt; the function text is UniProt's, condensed and in UK spelling. Roles: IntOGen calls it an activating (Act) driver in 1 cohort; IntOGen calls it a loss-of-function (LoF) driver in 2 cohorts; CIViC holds 1 clinical evidence items on its variants; UniProt disease notes describe a translocation or gene fusion involving the gene. Evidence tier "clinical-evidence" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.
- Lymphoma, t(11;18) API2-MALT1 and the NF-kB lesions of marginal zone lymphoma: Gastric MALT lymphoma begins as a Helicobacter-driven proliferation that still needs the antigen; removing the bacterium removes the stimulus and the lymphoma regresses. The t(11;18) fuses API2 to MALT1 and produces a protein that activates NF-kB on its own, so the lymphoma no longer needs the antigen and no longer cares whether the bacterium is still there. The related translocations, t(1;14) involving BCL10 and t(14;18) involving MALT1, do the same job by a different route, and inactivation of TNFAIP3 removes the brake. Frequency: Among 111 patients with Helicobacter-positive gastric MALT lymphoma treated with antibiotics, the translocation separated the responders from the non-responders: 47 of the 48 patients who regressed completely were negative for the API2-MALT1 transcript (Liu 2002). What it changes about treatment: Yes, and this is one of the few places where a translocation result changes the first decision. A t(11;18)-positive gastric MALT lymphoma should not be treated with eradication alone, whatever the stage; a negative one usually can be.

## Sources

- HGNC HGNC:989: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:989
- UniProt O95999: https://www.uniprot.org/uniprotkb/O95999/entry
- NCBI Gene 8915: https://www.ncbi.nlm.nih.gov/gene/8915
- Ensembl ENSG00000142867: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000142867
- Liu et al., Gastroenterology 2002: t(11;18) marks gastric MALT lymphomas that will not respond to Helicobacter pylori eradication (111 patients): https://doi.org/10.1053/gast.2002.33047

## Connected records

- collections: [CIViC](https://onco.cc/collections/civic/), [IntOGen](https://onco.cc/collections/intogen/), [Open Targets Platform](https://onco.cc/collections/open-targets/)
- cancers: [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/), [Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)](https://onco.cc/cancers/malt-lymphoma/), [Lung cancer (all types)](https://onco.cc/cancers/lung-cancer/), [Marginal zone lymphoma](https://onco.cc/cancers/marginal-zone-lymphoma/), [Non-Hodgkin lymphoma (all types)](https://onco.cc/cancers/non-hodgkin-lymphoma/)
- pathways: [B-cell receptor / BTK signalling (to NF-κB)](https://onco.cc/pathways/bcr-signalling/), [Inflammation & NF-κB](https://onco.cc/pathways/inflammation-nfkb/), [Microbiome-tumour interactions](https://onco.cc/pathways/microbiome-tumour/)

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JSON: https://onco.cc/api/v1/entities/bcl10.json