Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Centogene AG - |
RCV001809248 | SCV002059747 | uncertain significance | Smith-Magenis syndrome | 2021-04-09 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001869599 | SCV002256483 | uncertain significance | not provided | 2021-06-16 | 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. Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Benign"; Align-GVGD: "Class C25"). This variant has not been reported in the literature in individuals with RAI1-related conditions. This variant is not present in population databases (ExAC no frequency). This sequence change replaces proline with alanine at codon 1900 of the RAI1 protein (p.Pro1900Ala). The proline residue is highly conserved and there is a small physicochemical difference between proline and alanine. |
Prevention |
RCV004743580 | SCV005343860 | uncertain significance | RAI1-related disorder | 2024-02-26 | no assertion criteria provided | clinical testing | The RAI1 c.5698C>G variant is predicted to result in the amino acid substitution p.Pro1900Ala. To our knowledge, this variant has not been reported in the literature or in a large population database, indicating this variant is rare. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |