Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
3billion | RCV001808892 | SCV002059125 | likely pathogenic | Developmental and epileptic encephalopathy 99 | 2022-01-03 | criteria provided, single submitter | clinical testing | Same nucleotide change resulting in same amino acid change has been previously reported to be associated with ATP1A3 related disorder (ClinVar ID: VCV000161141, PMID:24523486, PS1_P). A different missense change at the same codon has been reported to be associated with ATP1A3 related disorder (PMID:24842602,25996915, PM5_P). In silico tool predictions suggest damaging effect of the variant on gene or gene product (REVEL: 0.949, 3CNET: 0.987, PP3_P). A missense variant is a common mechanism associated with Developmental and epileptic encephalopathy 99 (PP2_P). It is not observed in the gnomAD v2.1.1 dataset (PM2_M). Therefore, this variant is classified as likely pathogenic according to the recommendation of ACMG/AMP guideline. |