Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
3billion, |
RCV001808838 | SCV002058990 | uncertain significance | Neuronal ceroid lipofuscinosis 8 northern epilepsy variant | 2022-01-03 | criteria provided, single submitter | clinical testing | The variantis observed at an extremely low frequency in the gnomAD v2.1.1 dataset (total allele frequency: 0.000012, PM2_M). In silico tool predictions suggest damaging effect of the variant on gene or gene product (REVEL: 0.869, 3CNET: 0.991, PP3_P). A missense variant is a common mechanism associated with Ceroid lipofuscinosis (PP2_P). Therefore, this variant is classified as uncertain significance according to the recommendation of ACMG/AMP guideline. |
Gene |
RCV004762185 | SCV005369100 | uncertain significance | not provided | 2023-06-25 | criteria provided, single submitter | clinical testing | Not observed at significant frequency in large population cohorts (gnomAD); 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 |