Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001380026 | SCV001577953 | pathogenic | Retinitis pigmentosa 12; Leber congenital amaurosis 8 | 2023-06-14 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. ClinVar contains an entry for this variant (Variation ID: 1068463). Disruption of this splice site has been observed in individuals with retinitis pigmentosa (PMID: 30902645). This variant is not present in population databases (gnomAD no frequency). This sequence change affects an acceptor splice site in intron 2 of the CRB1 gene. It is expected to disrupt RNA splicing. Variants that disrupt the donor or acceptor splice site typically lead to a loss of protein function (PMID: 16199547), and loss-of-function variants in CRB1 are known to be pathogenic (PMID: 10508521, 22065545, 23379534, 25412400, 26957898, 28041643, 29391521). |
DBGen Ocular Genomics | RCV001587386 | SCV001816091 | pathogenic | Retinitis pigmentosa 12 | 2021-06-04 | criteria provided, single submitter | clinical testing | |
Genome- |
RCV003319471 | SCV004172591 | pathogenic | Leber congenital amaurosis 8 | 2023-04-11 | criteria provided, single submitter | clinical testing | |
Genome- |
RCV001587386 | SCV004172592 | pathogenic | Retinitis pigmentosa 12 | 2023-04-11 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV003319471 | SCV005058567 | likely pathogenic | Leber congenital amaurosis 8 | 2023-11-20 | criteria provided, single submitter | clinical testing | |
Laboratory of Genetics in Ophthalmology, |
RCV003319471 | SCV004023373 | pathogenic | Leber congenital amaurosis 8 | no assertion criteria provided | research |