Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001036941 | SCV001200331 | uncertain significance | Hypoplastic enamel-onycholysis-hypohidrosis syndrome | 2019-01-26 | criteria provided, single submitter | clinical testing | 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. Experimental studies and prediction algorithms are not available for this variant, and the functional significance of the affected amino acid(s) is currently unknown. This variant has not been reported in the literature in individuals with MSX1-related conditions. This variant is not present in population databases (ExAC no frequency). This sequence change results in a premature translational stop signal in the MSX1 gene (p.Lys228Glufs*111). While this is not anticipated to result in nonsense mediated decay, it is expected to disrupt the last 76 amino acids of the MSX1 protein. |
Institute of Human Genetics, |
RCV002222195 | SCV002499686 | uncertain significance | Tooth agenesis, selective, 1 | 2022-04-04 | criteria provided, single submitter | clinical testing | ACMG categories: PVS1 |