Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000183687 | SCV000236156 | likely benign | not provided | 2019-02-27 | criteria provided, single submitter | clinical testing | This variant is associated with the following publications: (PMID: 31983221) |
Labcorp Genetics |
RCV000463861 | SCV000549233 | uncertain significance | Dilated cardiomyopathy 1CC; Hypertrophic cardiomyopathy 20 | 2023-05-23 | 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. ClinVar contains an entry for this variant (Variation ID: 201945). This variant has not been reported in the literature in individuals affected with NEXN-related conditions. This variant is present in population databases (rs769530172, gnomAD 0.006%). This variant, c.1680_1682del, results in the deletion of 1 amino acid(s) of the NEXN protein (p.Glu562del), but otherwise preserves the integrity of the reading frame. |
Ambry Genetics | RCV002399679 | SCV002712834 | likely benign | Cardiovascular phenotype | 2021-03-22 | criteria provided, single submitter | clinical testing | This alteration is classified as likely 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. |
ARUP Laboratories, |
RCV000183687 | SCV004565000 | uncertain significance | not provided | 2023-06-15 | criteria provided, single submitter | clinical testing | The NEXN c.1680_1682del; p.Glu562del variant (rs397517848) is reported in the literature in an individual affected with dilated cardiomyopathy (Mazzarotto 2020). This variant is reported in ClinVar (Variation ID: 201945) and is found in the general population with an overall allele frequency of 0.0018% (5/279,708 alleles) in the Genome Aggregation Database. This variant deletes a single glutamate residue leaving the rest of the protein in-frame. Due to limited information, the clinical significance of this variant is uncertain at this time. References: Mazzarotto F et al. Reevaluating the Genetic Contribution of Monogenic Dilated Cardiomyopathy. Circulation. 2020 Feb 4;141(5):387-398. PMID: 31983221. |