Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
DBGen Ocular Genomics | RCV001591783 | SCV001815874 | likely pathogenic | Retinitis pigmentosa | 2021-06-16 | criteria provided, single submitter | clinical testing | |
3billion | RCV002283553 | SCV002573099 | likely pathogenic | Cone dystrophy 3 | 2024-07-30 | criteria provided, single submitter | clinical testing | The variant is not observed in the gnomAD v4.0.0 dataset. Missense changes are a common disease-causing mechanism. In silico tool predictions suggest damaging effect of the variant on gene or gene product [REVEL: 0.88 (>=0.6); 3Cnet: 0.74 (>=0.6)]. The same nucleotide change resulting in the same amino acid change has been previously reported to be associated with GUCA1A related disorder (ClinVar ID: VCV001213842). A different missense change at the same codon (p.Asp144Gly) has been reported to be associated with GUCA1A-related disorder (ClinVar ID: VCV000974934 / PMID: 32025184). Therefore, this variant is classified as likely pathogenic according to the recommendation of ACMG/AMP guideline. |