BCL10

Overview

BCL10 (B-cell CLL/lymphoma 10) is a signaling adapter protein that positively regulates NF-kB activation downstream of antigen receptor signaling. It forms a complex with MALT1 and CARD11 (the CBM complex) that is central to lymphocyte activation. Truncating mutations that preserve or alter the C-terminal PEST domain can produce oncogenic, constitutively active forms that drive NF-kB signaling in B-cell lymphomas.

Alterations observed in the corpus

  • Nonsense (truncating) mutations identified as a recurrent alteration in DLBCL and FL by whole-genome/exome sequencing of non-Hodgkin lymphomas; truncated products act as NF-kB positive regulators with oncogenic potential PMID:21796119
  • Implicated in one or two PCNSL cases as part of focal amplification or homozygous deletion events in genome-wide analysis of 18 PCNSL samples PMID:25991819

Cancer types (linked)

  • DLBCL / FL: Recurrent truncating mutations drive constitutive NF-kB signaling; identified alongside CARD11, CD79B, and MYD88 mutations in the BCR/NF-kB pathway PMID:21796119

Co-occurrence and mutual exclusivity

Therapeutic relevance

  • NF-kB pathway activation via CBM complex (CARD11–BCL10–MALT1) is a potential target for MALT1 protease inhibitors in ABC-DLBCL.

Open questions

  • Frequency of BCL10 mutations relative to CARD11 and MYD88 mutations in ABC vs GCB DLBCL subtypes requires further stratification.

Sources

This page was processed by crosslinker on 2026-05-14. - PMID:25991819

This page was processed by crosslinker on 2026-05-14.