Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003677664 | SCV004407746 | uncertain significance | not provided | 2022-11-07 | criteria provided, single submitter | clinical testing | This variant is not present in population databases (gnomAD no frequency). This variant results in the deletion of part of exon 6 (c.670-24_690delins36) of the ELOVL4 gene. While this variant is not anticipated to result in nonsense mediated decay, it likely alters RNA splicing and results in a disrupted protein product. This variant has not been reported in the literature in individuals affected with ELOVL4-related conditions. Variants that disrupt the consensus splice site are a relatively common cause of aberrant splicing (PMID: 17576681, 9536098). In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
3billion | RCV005254853 | SCV005904781 | likely pathogenic | Stargardt disease 3 | 2023-11-24 | criteria provided, single submitter | clinical testing | The variant is not observed in the gnomAD v2.1.1 dataset. Predicted Consequence/Location: Frameshift - predicted to result in a loss or disruption of normal protein function through protein truncation. The predicted truncated protein may be shortened by more than 10%. Therefore, the variant is classified as likely pathogenic according to the recommendation of ACMG/AMP guideline. |