Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ce |
RCV001091627 | SCV001247777 | pathogenic | not provided | 2017-07-01 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV004756168 | SCV005359082 | uncertain significance | SLC22A12-related disorder | 2024-09-20 | no assertion criteria provided | clinical testing | The SLC22A12 c.1096G>C variant is predicted to result in the amino acid substitution p.Gly366Arg. This variant was reported in compound heterozygous state in an individual with renal hypouricaemia (Stiburkova et al. 2013. PubMed ID: 23386035). Functional studies showed significantly decreased urate uptake, a mis-localized URAT1 signal and revealed a dominant negative effect by causing ER retention of both mutant and wild type proteins (Stiburkova et al. 2013. PubMed ID: 23386035; Stiburkova et al 2015. PubMed ID: 26418379). This variant is reported in 0.0018% of alleles in individuals of European (Non-Finnish) descent in gnomAD. Although we suspect this variant could be pathogenic, its significance remains uncertain at the moment. |