Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002543708 | SCV001507533 | uncertain significance | Catecholaminergic polymorphic ventricular tachycardia 1 | 2021-08-12 | criteria provided, single submitter | clinical testing | This sequence change replaces aspartic acid with glutamic acid at codon 379 of the CASQ2 protein (p.Asp379Glu). The aspartic acid residue is highly conserved and there is a small physicochemical difference between aspartic acid and glutamic acid. This variant is not present in population databases (ExAC no frequency). This variant has not been reported in the literature in individuals affected with CASQ2-related conditions. Algorithms developed to predict the effect of missense changes on protein structure and function output the following: SIFT: "Deleterious"; PolyPhen-2: "Not Available"; Align-GVGD: "Class C0". The glutamic acid amino acid residue is found in multiple mammalian species, which suggests that this missense change does not adversely affect protein function. 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 | RCV004034407 | SCV003959558 | uncertain significance | Cardiovascular phenotype | 2023-03-22 | criteria provided, single submitter | clinical testing | The c.1137T>G (p.D379E) alteration is located in exon 11 (coding exon 11) of the CASQ2 gene. This alteration results from a T to G substitution at nucleotide position 1137, causing the aspartic acid (D) at amino acid position 379 to be replaced by a glutamic acid (E). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |
Genome- |
RCV003346457 | SCV004050759 | uncertain significance | Catecholaminergic polymorphic ventricular tachycardia 2 | 2023-04-11 | criteria provided, single submitter | clinical testing |