Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
3billion | RCV002250846 | SCV002520968 | uncertain significance | Combined oxidative phosphorylation defect type 8 | 2022-05-22 | criteria provided, single submitter | clinical testing | The variant is observed at an extremely low frequency in the gnomAD v2.1.1 dataset (total allele frequency: 0.002%). In silico tool predictions suggest damaging effect of the variant on gene or gene product (REVEL: 0.92; 3Cnet: 0.98). A different missense change at the same codon (p.Arg625His) has been reported to be associated with AARS2 related disorder (ClinVar ID: VCV001207958). However the evidence of pathogenicity is insufficient at this time. Therefore, this variant is classified as uncertain significanceaccording to the recommendation of ACMG/AMP guideline. |
Prevention |
RCV004738564 | SCV005347985 | uncertain significance | AARS2-related disorder | 2024-07-10 | no assertion criteria provided | clinical testing | The AARS2 c.1873C>T variant is predicted to result in the amino acid substitution p.Arg625Cys. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.0056% of alleles in individuals of Latino descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |