Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Centre for Mendelian Genomics, |
RCV001196322 | SCV001366913 | likely pathogenic | Retinitis pigmentosa 51 | 2019-08-22 | criteria provided, single submitter | clinical testing | This variant was classified as: Likely pathogenic. The following ACMG criteria were applied in classifying this variant: PVS1,PM2. |
Labcorp Genetics |
RCV001863105 | SCV002207296 | pathogenic | Bardet-Biedl syndrome | 2024-01-29 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Met295Ilefs*15) in the TTC8 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in TTC8 are known to be pathogenic (PMID: 16308660, 16877420, 19797195, 21052717, 30886724). This variant is present in population databases (no rsID available, gnomAD 0.003%). This variant has not been reported in the literature in individuals affected with TTC8-related conditions. ClinVar contains an entry for this variant (Variation ID: 930561). For these reasons, this variant has been classified as Pathogenic. |
Baylor Genetics | RCV001196322 | SCV004207454 | likely pathogenic | Retinitis pigmentosa 51 | 2023-07-02 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV003918771 | SCV004732124 | pathogenic | TTC8-related disorder | 2024-03-29 | no assertion criteria provided | clinical testing | The TTC8 c.915delG variant is predicted to result in a frameshift and premature protein termination (p.Met305Ilefs*15). To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.0026% of alleles in individuals of European (non-Finnish) descent in gnomAD. Frameshift variants in TTC8 are expected to be pathogenic. This variant is interpreted as pathogenic. |