Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Diagnostics Services |
RCV002465077 | SCV002759472 | uncertain significance | Familial juvenile hyperuricemic nephropathy type 1 | 2022-10-20 | criteria provided, single submitter | clinical testing | The c.176A>G variant is not present in publicly available population databases like 1000 Genomes, EVS, ExAC, gnomAD, Indian Exome Database or our in-house exome database. This variant has neither been published nor reported to clinical databases like ClinVar, Human Genome Mutation Database (HGMD) or OMIM in any affected individuals. In-silico pathogenicity prediction programs like SIFT, PolyPhen-2, MutationTaster2, CADD etc. predicted this variant to be likely deleterious, however these predictions were not confirmed by any published functional studies. The variant is located in a mutational hotspot region and an alternative variant in this position (c.176A>C, p.Asp59Ala) has been previously identified in similarly affected individuals (PMID: 14569098) and reported to HGMD (ID: CM034982). |