Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001369460 | SCV001565901 | uncertain significance | Dyskeratosis congenita, autosomal dominant 6 | 2021-10-19 | 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 (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be tolerated. This variant has not been reported in the literature in individuals affected with ACD-related conditions. This variant is present in population databases (rs779041176, ExAC 0.006%). This sequence change replaces alanine with serine at codon 499 of the ACD protein (p.Ala499Ser). The alanine residue is moderately conserved and there is a moderate physicochemical difference between alanine and serine. |
Ambry Genetics | RCV002395837 | SCV002702011 | uncertain significance | Inborn genetic diseases | 2021-11-02 | criteria provided, single submitter | clinical testing | The p.A499S variant (also known as c.1495G>T), located in coding exon 11 of the ACD gene, results from a G to T substitution at nucleotide position 1495. The alanine at codon 499 is replaced by serine, an amino acid with similar properties. This amino acid position is conserved. In addition, this alteration is predicted to be tolerated by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |