Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV000213648 | SCV000274677 | likely pathogenic | Hereditary cancer-predisposing syndrome | 2015-03-16 | criteria provided, single submitter | clinical testing | The c.48+1G>C intronic variant results from a G to C substitution one nucleotide after coding exon 1 of the PALB2 gene. This variant was first reported in an individual with breast cancer and family history of breast cancer (Hellebrand H, et al. Hum. Mutat. 2011 Jun; 32(6):E2176-88). Using the BDGP and ESEfinder splice site prediction tools, this alteration is predicted to abolish the native donor splice site; however, direct evidence is unavailable. Alterations that disrupt the canonical splice site are expected to cause aberrant splicing, resulting in an abnormal protein or a transcript that is subject to nonsense-mediated mRNA decay. As such, this alteration is classified as likely pathogenic. |
Gene |
RCV000236723 | SCV000293389 | uncertain significance | not provided | 2015-12-22 | criteria provided, single submitter | clinical testing | This variant is denoted PALB2 c.48+1G>C or IVS1+1G>C and consists of a G>C nucleotide substitution at the +1 position of intron 1 of the PALB2 gene. This variant destroys a canonical splice donor site and is predicted to cause skipping of exon 2, resulting in an in-frame deletion of 20 amino acids that are not part of a critical functional domain. This variant has been reported in an individual with a personal and family history of breast cancer (Hellebrand 2011). Based on the current evidence, we consider PALB2 c.48+1G>C to be a variant of uncertain significance. |
Myriad Genetics, |
RCV001030106 | SCV004019437 | likely pathogenic | Familial cancer of breast | 2023-03-01 | criteria provided, single submitter | clinical testing | This variant is considered likely pathogenic. This variant occurs within a consensus splice junction and is predicted to result in abnormal mRNA splicing of either an out-of-frame exon or an in-frame exon necessary for protein stability and/or normal function. |
Leiden Open Variation Database | RCV001030106 | SCV001192906 | likely pathogenic | Familial cancer of breast | 2019-05-13 | no assertion criteria provided | curation | Curators: Marc Tischkowitz, Arleen D. Auerbach. Submitters to LOVD: Alfons Meindl, Marc Tischkowitz. |