Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000413987 | SCV000491533 | likely pathogenic | not provided | 2016-06-28 | criteria provided, single submitter | clinical testing | The c.3339+5G>A variant in the RPGRIP1 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. This splice site variant destroys the canonical splice donor site in intron 20, and splice predictor models indicate this variant creates a cryptic splice donor site downstream of the natural splice donor site in intron 20. It is expected to cause abnormal gene splicing. The c.3339+5G>A variant was not observed in approximately 6300 individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, indicating it is not a common benign variant in these populations. The c.3339+5G>A variant is a strong candidate for a pathogenic variant, however the possibility it may be a rare benign variant cannot be excluded. |
Rui Chen Lab, |
RCV000515742 | SCV000579408 | pathogenic | Leber congenital amaurosis | 2017-05-09 | no assertion criteria provided | research |