Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV000621473 | SCV000736252 | uncertain significance | Cardiovascular phenotype | 2023-12-06 | criteria provided, single submitter | clinical testing | The p.S1795T variant (also known as c.5383T>A), located in coding exon 24 of the DSP gene, results from a T to A substitution at nucleotide position 5383. The serine at codon 1795 is replaced by threonine, an amino acid with similar properties. This amino acid position is highly conserved in available vertebrate species. In addition, the in silico prediction for this alteration is inconclusive. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Color Diagnostics, |
RCV001190257 | SCV001357708 | uncertain significance | Cardiomyopathy | 2023-07-28 | criteria provided, single submitter | clinical testing | This missense variant replaces serine with threonine at codon 1795 of the DSP protein. Computational prediction suggests that this variant may not impact protein structure and function (internally defined REVEL score threshold <= 0.5, PMID: 27666373). To our knowledge, functional studies have not been performed for this variant. This variant has been reported in an individual affected with arrhythmogenic right ventricular cardiomyopathy (PMID: 32659924). This variant has been identified in 3/282582 chromosomes in the general population by the Genome Aggregation Database (gnomAD). The available evidence is insufficient to determine the role of this variant in disease conclusively. Therefore, this variant is classified as a Variant of Uncertain Significance. |
Labcorp Genetics |
RCV001304150 | SCV001493421 | likely benign | Arrhythmogenic cardiomyopathy with wooly hair and keratoderma; Arrhythmogenic right ventricular dysplasia 8 | 2024-01-29 | criteria provided, single submitter | clinical testing | |
All of Us Research Program, |
RCV004002656 | SCV004829695 | uncertain significance | Arrhythmogenic cardiomyopathy with wooly hair and keratoderma | 2023-08-08 | criteria provided, single submitter | clinical testing | This missense variant replaces serine with threonine at codon 1795 of the DSP protein. Computational prediction suggests that this variant may not impact protein structure and function (internally defined REVEL score threshold <= 0.5, PMID: 27666373). To our knowledge, functional studies have not been performed for this variant. This variant has been reported in an individual affected with arrhythmogenic right ventricular cardiomyopathy (PMID: 32659924). This variant has been identified in 3/282582 chromosomes in the general population by the Genome Aggregation Database (gnomAD). The available evidence is insufficient to determine the role of this variant in disease conclusively. Therefore, this variant is classified as a Variant of Uncertain Significance. |