Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
3billion | RCV001808119 | SCV002058605 | pathogenic | Crigler-Najjar syndrome, type II | 2022-01-03 | criteria provided, single submitter | clinical testing | Functional studies provide strong evidence of the variant having a damaging effect on the gene or gene product (PMID:7852413, PS3_S). In silico tool predictions suggest damaging effect of the variant on gene or gene product (REVEL: 0.922, 3CNET: 0.985, PP3_P). A missense variant is a common mechanism associated with Crigler-Najjar syndrome (PP2_P). It is observed at an extremely low frequency in the gnomAD v2.1.1 dataset (total allele frequency: 0.000008, PM2_M). Therefore, this variant is classified as pathogenic according to the recommendation of ACMG/AMP guideline. |