Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001863073 | SCV002289561 | uncertain significance | Fanconi anemia | 2021-10-14 | criteria provided, single submitter | clinical testing | This sequence change replaces alanine with valine at codon 1694 of the SLX4 protein (p.Ala1694Val). The alanine residue is weakly conserved and there is a small physicochemical difference between alanine and valine. This variant is present in population databases (rs761226343, ExAC 0.009%). This missense change has been observed in individual(s) with breast cancer (PMID: 22911665). ClinVar contains an entry for this variant (Variation ID: 929617). Algorithms developed to predict the effect of missense changes on protein structure and function (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be tolerated. 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. |
Prevention |
RCV003425982 | SCV004117462 | uncertain significance | SLX4-related disorder | 2023-08-03 | criteria provided, single submitter | clinical testing | The SLX4 c.5081C>T variant is predicted to result in the amino acid substitution p.Ala1694Val. This variant has been reported in patient with familial breast cancer (Table S1A, Bakker et al. 2012. PubMed ID: 22911665). This variant is reported in 0.0028% of alleles in individuals of Latino descent in gnomAD (http://gnomad.broadinstitute.org/variant/16-3633170-G-A). At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |
Leiden Open Variation Database | RCV001194868 | SCV001364705 | likely benign | not specified | 2012-08-31 | no assertion criteria provided | curation | Curator: Arleen D. Auerbach. Submitter to LOVD: Janine Bakker. |