Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Rare Kidney Stone Consortium and the Mayo Clinic Hyperoxaluria Center, |
RCV003445259 | SCV004171761 | likely pathogenic | Primary hyperoxaluria, type I | 2023-10-27 | criteria provided, single submitter | clinical testing | ACMG:PVS1 PM2 PP4 PP5 |
Department of Genetics, |
RCV003445259 | SCV005387814 | pathogenic | Primary hyperoxaluria, type I | 2024-10-31 | no assertion criteria provided | clinical testing | The c.34_35dup pathogenic mutation, located in the first exon of the AGXT gene, causing a translational frameshift with a predicted alternate stop codon (p.Ala13Argfs*34). This mutation has been identified in individual(s) with primary Hyperoxaluria type 1 (PMID:25644115). This variant is not present in population databases (gnomAD no frequency). ClinVar contains an entry for this variant (Variation ID:2664113). For these reasons, this variant has been classified as Pathogenic. |