Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003770304 | SCV004606786 | pathogenic | Cone-rod dystrophy 6; Leber congenital amaurosis 1 | 2023-11-28 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Phe999Leufs*22) in the GUCY2D gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in GUCY2D are known to be pathogenic (PMID: 10951519, 11328726). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with GUCY2D-related conditions. ClinVar contains an entry for this variant (Variation ID: 974653). For these reasons, this variant has been classified as Pathogenic. |
Laboratory of Genetics in Ophthalmology, |
RCV001250852 | SCV001426344 | pathogenic | Leber congenital amaurosis 1 | no assertion criteria provided | research |