Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV002364144 | SCV002659269 | pathogenic | Diamond-Blackfan anemia | 2020-10-21 | criteria provided, single submitter | clinical testing | The c.65_c.75+2del13insCTGG variant results from a deletion of 13 nucleotides and insertion of 4 nucleotides between positions 65 and 75+2 and involves the canonical splice donor site after coding exon 1 of the RPS7 gene. The canonical splice donor site is highly conserved in available vertebrate species. 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 interpreted as a disease-causing mutation. |