Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000637321 | SCV000758772 | uncertain significance | Hereditary breast ovarian cancer syndrome | 2023-02-01 | criteria provided, single submitter | clinical testing | ClinVar contains an entry for this variant (Variation ID: 531202). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with BRCA1-related conditions. This sequence change falls in intron 8 of the BRCA1 gene. It does not directly change the encoded amino acid sequence of the BRCA1 protein. It affects a nucleotide within the consensus splice site. Variants that disrupt the consensus splice site are a relatively common cause of aberrant splicing (PMID: 17576681, 9536098). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
Gene |
RCV001766360 | SCV002001007 | uncertain significance | not provided | 2020-05-01 | criteria provided, single submitter | clinical testing | Has not been previously published as pathogenic or benign to our knowledge; Not observed in large population cohorts (Lek 2016); In silico analysis supports a deleterious effect on splicing; Also known as 712+4delG |
Ambry Genetics | RCV002358790 | SCV002653947 | uncertain significance | Hereditary cancer-predisposing syndrome | 2024-02-27 | criteria provided, single submitter | clinical testing | The c.593+4delA intronic variant, located in intron 7 of the BRCA1 gene, results from a deletion of one nucleotide within intron 7 of the BRCA1 gene. This nucleotide position is highly conserved in available vertebrate species. In silico splice site analysis predicts that this alteration will weaken the native splice donor site; however, this alteration occurs at a splice junction that is located at a naturally occurring, alternatively spliced exon and, thus, the effects of abnormal splicing cannot be predicted (Ambry internal data; Colombo M et al. Hum. Mol. Genet., 2014 Jul;23:3666-80). Based on the available evidence, the clinical significance of this alteration remains unclear. |
Baylor Genetics | RCV003459523 | SCV004215064 | likely pathogenic | Breast-ovarian cancer, familial, susceptibility to, 1 | 2023-11-27 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV005019057 | SCV005647188 | uncertain significance | Breast-ovarian cancer, familial, susceptibility to, 1; Pancreatic cancer, susceptibility to, 4; Fanconi anemia, complementation group S | 2024-02-15 | criteria provided, single submitter | clinical testing |