Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Invitae | RCV000631711 | SCV000752798 | uncertain significance | Long QT syndrome | 2023-11-14 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with cysteine, which is neutral and slightly polar, at codon 3233 of the AKAP9 protein (p.Arg3233Cys). This variant is present in population databases (rs61757674, gnomAD 0.03%). This variant has not been reported in the literature in individuals affected with AKAP9-related conditions. ClinVar contains an entry for this variant (Variation ID: 527014). An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be disruptive. 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. |
Ambry Genetics | RCV002385983 | SCV002694254 | benign | Cardiovascular phenotype | 2024-01-08 | criteria provided, single submitter | clinical testing | This alteration is classified as benign based on a combination of the following: seen in unaffected individuals, population frequency, intact protein function, lack of segregation with disease, co-occurrence, RNA analysis, in silico models, amino acid conservation, lack of disease association in case-control studies, and/or the mechanism of disease or impacted region is inconsistent with a known cause of pathogenicity. |
Fulgent Genetics, |
RCV002477384 | SCV002787026 | uncertain significance | Long QT syndrome 11 | 2022-02-09 | criteria provided, single submitter | clinical testing | |
Dept of Medical Biology, |
RCV000631711 | SCV004021968 | uncertain significance | Long QT syndrome | 2024-01-08 | criteria provided, single submitter | research | Criteria: BP4 |
Ce |
RCV003884674 | SCV004698328 | likely benign | not provided | 2024-02-01 | criteria provided, single submitter | clinical testing | AKAP9: BP4 |