Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
University of Washington Department of Laboratory Medicine, |
RCV004731686 | SCV005331510 | uncertain significance | Developmental and epileptic encephalopathy 104; Neurodevelopmental disorder with epilepsy and brain atrophy | 2023-08-01 | criteria provided, single submitter | clinical testing | The p.Asp96Val variant in the ATP6V0A1 gene has not been previously reported in association with disease and was absent from large population databases, including the Genome Aggregation Database (http://gnomad.broadinstitute.org/). The ATP6V0A1 gene has fewer missense variants in the general population than expected. A low rate of missense variation may suggest that this gene is intolerant to missense variation. In silico tools predict that the p.Asp96Val variant is deleterious; however, these predictions have not been tested directly. Using ACMG guidelines, this variant was classified as a variant of uncertain significance (ACMG evidence codes used: PM2_supporting, PP2, PP3). |