Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000808539 | SCV000948649 | uncertain significance | Long QT syndrome | 2022-11-15 | criteria provided, single submitter | clinical testing | 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. 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. ClinVar contains an entry for this variant (Variation ID: 652879). This variant has not been reported in the literature in individuals affected with AKAP9-related conditions. This variant is present in population databases (rs779260877, gnomAD 0.004%). This sequence change replaces glutamine, which is neutral and polar, with glutamic acid, which is acidic and polar, at codon 3032 of the AKAP9 protein (p.Gln3032Glu). |
Ambry Genetics | RCV002370160 | SCV002684699 | uncertain significance | Cardiovascular phenotype | 2023-12-16 | criteria provided, single submitter | clinical testing | The p.Q3032E variant (also known as c.9094C>G), located in coding exon 37 of the AKAP9 gene, results from a C to G substitution at nucleotide position 9094. The glutamine at codon 3032 is replaced by glutamic acid, an amino acid with highly similar properties. This amino acid position is not well conserved in available vertebrate species. In addition, this alteration is predicted to be tolerated by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Fulgent Genetics, |
RCV002487742 | SCV002777352 | uncertain significance | Long QT syndrome 11 | 2021-08-18 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV003411786 | SCV004113929 | uncertain significance | AKAP9-related disorder | 2022-09-14 | criteria provided, single submitter | clinical testing | The AKAP9 c.9094C>G variant is predicted to result in the amino acid substitution p.Gln3032Glu. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.0040% of alleles in individuals of African descent in gnomAD (http://gnomad.broadinstitute.org/variant/7-91715611-C-G). At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |