Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Centre for Mendelian Genomics, |
RCV000415295 | SCV000493035 | likely pathogenic | Cardiac arrhythmia; Noncompaction cardiomyopathy | 2013-12-13 | criteria provided, single submitter | clinical testing | |
Centre for Mendelian Genomics, |
RCV001214045 | SCV001368765 | likely pathogenic | Left ventricular noncompaction 1 | 2016-01-01 | criteria provided, single submitter | clinical testing | This variant was classified as: Likely pathogenic. |
Labcorp Genetics |
RCV001214045 | SCV001385708 | uncertain significance | Left ventricular noncompaction 1 | 2019-09-13 | criteria provided, single submitter | clinical testing | This sequence change replaces isoleucine with methionine at codon 59 of the DTNA protein (p.Ile59Met). The isoleucine residue is highly conserved and there is a small physicochemical difference between isoleucine and methionine. 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 are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C0"). This variant has been reported in individuals in the Leiden Open-source Variation Database (PMID: 21520333). ClinVar contains an entry for this variant (Variation ID: 374197). This variant is not present in population databases (ExAC no frequency). |