Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
3billion | RCV001808297 | SCV002058968 | pathogenic | Renal hypomagnesemia 5 with ocular involvement | 2022-01-03 | criteria provided, single submitter | clinical testing | Frameshift: predicted to result in a loss or disruption of normal protein function through nonsense-mediated decay (NMD) or protein truncation. Multiple pathogenic variants are reported downstream of the variant (PVS1_VS). The variant has been reported to be associated with CLDN19 related disorder (3billion dataset). It is not observed in the gnomAD v2.1.1 dataset (PM2_M). Therefore, this variant is classified as pathogenic according to the recommendation of ACMG/AMP guideline. |
Labcorp Genetics |
RCV002542434 | SCV002955540 | pathogenic | not provided | 2022-08-22 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 1333609). This variant has not been reported in the literature in individuals affected with CLDN19-related conditions. This variant is not present in population databases (gnomAD no frequency). This sequence change creates a premature translational stop signal (p.Leu143Trpfs*2) in the CLDN19 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in CLDN19 are known to be pathogenic (PMID: 22422540). |