Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Color Diagnostics, |
RCV000772520 | SCV000905700 | pathogenic | Hereditary cancer-predisposing syndrome | 2020-04-21 | criteria provided, single submitter | clinical testing | This variant deletes 4 nucleotides in exon 14 of the BAP1 gene, creating a frameshift and premature translation stop signal. This variant is expected to result in an absent or non-functional protein product. This variant has been reported in individuals affected with melanoma, colorectal cancer, renal cell carcinoma, breast cancer, and malignant mesothelioma (PMID: 24243779, 26719535). This variant has not been identified in the general population by the Genome Aggregation Database (gnomAD). Loss of BAP1 function is a known mechanism of disease (clinicalgenome.org). Based on the available evidence, this variant is classified as Pathogenic. |
Ce |
RCV001312026 | SCV001502442 | pathogenic | not provided | 2022-08-01 | criteria provided, single submitter | clinical testing | BAP1: PVS1, PM2 |
Labcorp Genetics |
RCV001850964 | SCV002120757 | pathogenic | BAP1-related tumor predisposition syndrome | 2024-09-24 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Ser628Profs*8) in the BAP1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in BAP1 are known to be pathogenic (PMID: 21874000, 23684012). This variant is not present in population databases (gnomAD no frequency). This premature translational stop signal has been observed in individual(s) with BAP1 tumor predisposition syndrome (PMID: 21874000, 24243779). ClinVar contains an entry for this variant (Variation ID: 628182). For these reasons, this variant has been classified as Pathogenic. |
OMIM | RCV001850964 | SCV000044530 | pathogenic | BAP1-related tumor predisposition syndrome | 2011-08-28 | no assertion criteria provided | literature only |