Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV001018804 | SCV001180083 | likely pathogenic | Hereditary cancer-predisposing syndrome | 2021-06-15 | criteria provided, single submitter | clinical testing | The c.908+3A>T intronic variant results from an A to T substitution 3 nucleotides after coding exon 7 in the CHEK2 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. RNA studies have demonstrated this alteration results in abnormal splicing in the set of samples tested (Ambry internal data). Based on the majority of available evidence to date, this variant is likely to be pathogenic. |
Labcorp Genetics |
RCV001059485 | SCV001224109 | uncertain significance | Familial cancer of breast | 2022-03-05 | criteria provided, single submitter | clinical testing | 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. Variants that disrupt the consensus splice site are a relatively common cause of aberrant splicing (PMID: 17576681, 9536098). Studies have shown this variant is associated with skipping of exon 8, but one or more of the resulting mRNA isoform(s) may be naturally occurring (PMID: 28905878; Invitae). ClinVar contains an entry for this variant (Variation ID: 822989). This variant has not been reported in the literature in individuals affected with CHEK2-related conditions. This variant is not present in population databases (gnomAD no frequency). This sequence change falls in intron 8 of the CHEK2 gene. It does not directly change the encoded amino acid sequence of the CHEK2 protein. It affects a nucleotide within the consensus splice site. |
Baylor Genetics | RCV001059485 | SCV004217548 | uncertain significance | Familial cancer of breast | 2023-09-19 | criteria provided, single submitter | clinical testing |