Gene

BAP1

BRCA1 associated deubiquitinase 1

Explore in graph →CI-GENE-00021620HGNC:950 ENSG00000163930 NCBI 8314 CIViC Cancer geneApproved

Curated evidence

Clinical evidence (19)

CIViC items involving this gene, grouped by molecular profile and therapy, with native levels and directions. 50 items per page.

civicCuratedShowing 1–19 of 19 evidence items · levels, directions and significance as curated at the source; each row links to its CIViC record.

Therapy	Cancer	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
BAP1 ALTERNATIVE TRANSCRIPT (ATI)1
Apitolisib + OlaparibCombination	Malignant Mesothelioma	Predictive	D	Supports Sensitivity Response	3

Cohorts

Alteration frequency by cohort (143)

Frequency = cases affected / cases profiled within one cohort. Cohorts are never pooled.

gdcObserved143 cohort rows (showing 50, sorted by frequency) · frequency = affected / profiled, as published by each cohort; cohorts are not pooled. Hover a row badge for its release.

Cohort	Mapped cancer	Alteration	Affected (n)	Profiled (n)	Frequency (%)	Rank in cohort	Source
Kidney Renal Clear Cell Carcinoma TCGA-KIRC	Clear Cell Renal Cell Carcinoma

Literature

Linked publications (8)

25 per page, newest first.

pubmedPublishedShowing 1–8 of 8 publications · bibliographic data as recorded by PubMed; abstracts are not reproduced.

Integrated molecular analysis of clear-cell renal cell carcinoma.
· Nat Genet · 2013 · PMID 23797736 · validated civic_curation pubmed
Comprehensive molecular characterization of clear cell renal cell carcinoma.
· Nature · 2013 · PMID 23792563 · validated civic_curation pubmed
Genomic Biomarkers of a Randomized Trial Comparing First-line Everolimus and Sunitinib in Patients with Metastatic Renal Cell Carcinoma.
· Eur Urol · 2017 · PMID 27751729 · validated civic_curation pubmed
Prognostic parameters in uveal melanoma and their association with BAP1 expression.
· Br J Ophthalmol · 2014 · PMID 25147369 · validated civic_curation pubmed

BAP1 c.122+1G>T1
(predisposing)	Meningioma	Predisposing	C	Supports Predisposition	2	submitted	EID13245 Jadun et al. reported an adolescent girl with a skull-base meningothelial meningioma, WHO grade 1. Initial germline testing of NF2, LZTR1, and SMARCB1 was negative. Subsequent paired tumor-normal geno… (full text at CIViC) PMID 41442315 · Jadun et al., 2026 · Open in CIViC	civic

BAP1 c.122+1G>T1

(predisposing)

Meningioma

Predisposing

Supports Predisposition

submitted

EID13245

Jadun et al. reported an adolescent girl with a skull-base meningothelial meningioma, WHO grade 1. Initial germline testing of NF2, LZTR1, and SMARCB1 was negative. Subsequent paired tumor-normal geno… (full text at CIViC)

PMID 41442315 · Jadun et al., 2026 · Open in CIViC

BAP1 Loss4
(functional)	—UNRESOLVED	Functional	D	Supports Gain Of Function	3	submitted	EID12007 In this paper, the collateral vulnerabilities that arise as a consequence of the inactivation or loss of function of certain tumour suppressor genes, specifically in malignant pleural mesothelioma (MP… (full text at CIViC) PMID 33072611 · Xu et al., 2020 · Open in CIViC	civic
EPZ011989	Pleural Malignant MesotheliomaALIAS	Predictive	D	Supports Sensitivity Response	2	submitted	EID8329 The in vivo experiment demonstrated that Bap1 loss results in increased trimethylated histone H3 lysine 27 (H3K27me3), elevated Ezh2 expression, and enhanced repression of Polycomb Repressive Complex … (full text at CIViC) PMID 26437366 · LaFave et al., 2015 · Open in CIViC	civic
Mocetinostat + VorinostatSubstitutes	Malignant Mesothelioma	Predictive	E	Supports Sensitivity Response	2	accepted	EID1235 A siRNA screen identified BAP1 associated with HDAC1 and HDAC2 expression. HDAC2 or BAP1 depletion sensitized a mesothelioma cell line (MSTO-211H) to HDAC inhibitors vorinostat or mocetinostat. The pa… (full text at CIViC) PMID 25970771 · Sacco et al., 2015 · Open in CIViC	civic
Olaparib	Kidney CarcinomaALIAS	Predictive	D	Supports Sensitivity Response	3	submitted	EID5930 BAP1 loss sensitized cells to the PARP inhibitor olaparib. In addition, BAP1-deficient cells were more sensitive to ionizing radiation. PMID 22683710 · Peña-Llopis et al., 2012 · Open in CIViC	civic

BAP1 Mutation8
(prognostic)	Clear Cell Renal Cell Carcinoma	Prognostic	B	Supports Poor Outcome	5	accepted	EID459 In patients with clear cell renal cell carcinoma, in a multivariate analysis those with mutations in BAP1 had worse overall survival than wild-type. PMID 23797736 · Sato et al., 2013 · Open in CIViC	civic
〃	Renal Cell CarcinomaCURATED_BROADER	Prognostic	B	Supports Poor Outcome	5	accepted	EID460 In patients with clear cell renal cell carcinoma, in a multivariate analysis those with mutations in BAP1 had worse overall survival than wild-type. PMID 23797736 · Sato et al., 2013 · Open in CIViC	civic
〃	Clear Cell Renal Cell Carcinoma	Prognostic	B	Supports Poor Outcome	5	accepted	EID461 In patients with clear cell renal cell carcinoma, those with nonsilent mutations of BAP1 had worse overall survival than wild-type. PMID 23792563 · 2013, Nature · Open in CIViC	civic
CDK4/6 Inhibition	Pleural Malignant MesotheliomaALIAS	Predictive	D	Supports Sensitivity Response	4	submitted	EID12008 The effect of CDK4/6 inhibitors (abemaciclib or palbociclib) was tested in patient-derived malignant pleural mesothelioma (MLM) cell lines. Of the three patient-derived cell lines, two of the cell lin… (full text at CIViC) PMID 34588615 · Aliagas et al., 2021 · Open in CIViC	civic
Everolimus + SunitinibSequential	Clear Cell Renal Cell Carcinoma	Predictive	B	Supports Resistance	4	accepted	EID5339 This study evaluated the effects of everolimus and sunitinib on patients with metastatic renal cell carcinoma. A total of 220 patients with RCC were evaluated for 341 genes at 540x coverage. Of all 22… (full text at CIViC) PMID 27751729 · Hsieh et al., 2017 · Open in CIViC	civic
Olaparib	Malignant Mesothelioma	Predictive	B	Supports Sensitivity Response	3	submitted	EID11740 In this study, 23 patients with refractory mesothelioma harbouring germline or somatic BAP1 mutations were treated with olaparib. The median PFS was 3.6 months (95% CI, 2.7-4.2), and the median OS was… (full text at CIViC) PMID 34661178 · Ghafoor et al., 2021 · Open in CIViC	civic
〃	Malignant Mesothelioma	Predictive	B	Supports Sensitivity Response	3	submitted	EID11741 In this study, 23 patients with refractory mesothelioma harbouring germline or somatic BAP1 mutations were treated with olaparib. The median PFS was 3.6 months (95% CI, 2.7-4.2), and the median OS was… (full text at CIViC) PMID 34661178 · Ghafoor et al., 2021 · Open in CIViC	civic
Panobinostat + Trichostatin A + Valproic Acid + VorinostatSubstitutes	Uveal Melanoma	Predictive	D	Supports Sensitivity Response	3	accepted	EID1234 Somatic and germline BAP1 mutations are associated with uveal melanoma. In this preclinical study, gene set enrichment analysis revealed HDAC inhibitors as potential drugs in BAP1 mutant uveal melanom… (full text at CIViC) PMID 22038994 · Landreville et al., 2012 · Open in CIViC	civic