Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV004584564 | SCV002578104 | pathogenic | See cases | 2021-12-15 | criteria provided, single submitter | clinical testing | ACMG categories: PVS1,PM2,PP3 |
Prevention |
RCV003408213 | SCV004107039 | uncertain significance | FAM111A-related disorder | 2022-11-21 | criteria provided, single submitter | clinical testing | The FAM111A c.364C>T variant is predicted to result in premature protein termination (p.Gln122*). To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.0039% of alleles in individuals of European (Non-Finnish) descent in gnomAD (http://gnomad.broadinstitute.org/variant/11-58919505-C-T). At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |
Labcorp Genetics |
RCV003738179 | SCV004553490 | uncertain significance | not provided | 2024-01-22 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Gln122*) in the FAM111A gene. While this is not anticipated to result in nonsense mediated decay, it is expected to disrupt the last 490 amino acid(s) of the FAM111A protein. This variant is present in population databases (no rsID available, gnomAD 0.004%). This variant has not been reported in the literature in individuals affected with FAM111A-related conditions. ClinVar contains an entry for this variant (Variation ID: 1708456). In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |