Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Baylor Genetics | RCV001332380 | SCV001524694 | likely pathogenic | Vici syndrome | 2019-12-09 | criteria provided, single submitter | clinical testing | This variant was determined to be likely pathogenic according to ACMG Guidelines, 2015 [PMID:25741868]. |
Labcorp Genetics |
RCV001332380 | SCV003442590 | pathogenic | Vici syndrome | 2023-09-03 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. ClinVar contains an entry for this variant (Variation ID: 1030744). This variant has not been reported in the literature in individuals affected with EPG5-related conditions. This variant is present in population databases (rs372940918, gnomAD 0.006%). This sequence change creates a premature translational stop signal (p.Arg241*) in the EPG5 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in EPG5 are known to be pathogenic (PMID: 23222957, 23674064). |