Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
3billion, |
RCV003314291 | SCV004013501 | likely pathogenic | X-linked Alport syndrome | criteria provided, single submitter | clinical testing | The variant is not observed in the gnomAD v2.1.1 dataset. The variant is located in a mutational hot spot and/or well-established functional domain in which established pathogenic variants have been reported (PMID: 27627812). In silico tool predictions suggest damaging effect of the variant on gene or gene product (REVEL: 0.91; 3Cnet: 0.97). Different nucleotide change resulting in same amino acid change has been previously reported to be associated with COL4A5 related disorder (ClinVar ID: VCV001495185). Therefore, this variant is classified as Likely pathogenic according to the recommendation of ACMG/AMP guideline. |