BRCC3
Overview
BRCC3 (BRCA1/BRCA2-Containing Complex, Subunit 3), also known as KIAA0157 or BRE, encodes a deubiquitinase subunit of the BRCC36 isopeptidase complex involved in DNA damage response. It is X-linked and has been identified as a recurrently mutated driver gene in chronic lymphocytic leukemia (CLL), where it contributes to the DNA damage response pathway alongside other drivers such as CHEK2, ELF4, and DYRK1A.
Alterations observed in the corpus
- BRCC3 identified as a DNA damage pathway driver in CLL in a 538-sample whole-exome sequencing study; classified alongside CHEK2, ELF4, and DYRK1A as part of the DNA damage response driver category in CLL PMID:26466571.
Cancer types (linked)
- CLLSLL: Recurrently mutated DNA damage driver in CLL; identified as part of the 44 putative driver genes in a 538-sample WES study (278 samples prospectively collected on the CLL8 frontline chemoimmunotherapy trial) PMID:26466571.
Co-occurrence and mutual exclusivity
- BRCC3 mutations occur within the broader landscape of 44 driver genes across 538 CLL cases; 91.1% of CLLs harbor at least one of 55 driver events including BRCC3 PMID:26466571.
Therapeutic relevance
- No specific targeted agents against BRCC3 are reported in this corpus. Its role in the DNA damage response pathway in CLL may have implications for the activity of genotoxic agents used in chemoimmunotherapy (fludarabine/cyclophosphamide ± rituximab) PMID:26466571.
Open questions
- The standalone mutation frequency for BRCC3 and its independent prognostic value for PFS in frontline CLL treatment are not individually quantified in the CLL8 trial data as reported in this corpus.
Sources
This page was processed by crosslinker on 2026-05-14.