Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000200509 | SCV000250735 | uncertain significance | not provided | 2020-02-21 | criteria provided, single submitter | clinical testing | In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Has not been previously published as pathogenic or benign to our knowledge |
Ambry Genetics | RCV002327037 | SCV002630483 | uncertain significance | Familial thoracic aortic aneurysm and aortic dissection | 2023-09-11 | criteria provided, single submitter | clinical testing | The p.Y139C variant (also known as c.416A>G), located in coding exon 2 of the SLC2A10 gene, results from an A to G substitution at nucleotide position 416. The tyrosine at codon 139 is replaced by cysteine, an amino acid with highly dissimilar properties. This amino acid position is conserved. In addition, this alteration is predicted to be deleterious by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Labcorp Genetics |
RCV002515368 | SCV003505536 | uncertain significance | Arterial tortuosity syndrome | 2022-07-02 | criteria provided, single submitter | clinical testing | This sequence change replaces tyrosine, which is neutral and polar, with cysteine, which is neutral and slightly polar, at codon 139 of the SLC2A10 protein (p.Tyr139Cys). This variant is present in population databases (rs139858464, gnomAD 0.04%). This variant has not been reported in the literature in individuals affected with SLC2A10-related conditions. ClinVar contains an entry for this variant (Variation ID: 213745). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt SLC2A10 protein function. Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. 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. |
Breakthrough Genomics, |
RCV000200509 | SCV005195016 | uncertain significance | not provided | criteria provided, single submitter | not provided |