Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Center for Human Genetics, |
RCV000498737 | SCV000579546 | uncertain significance | Primary dilated cardiomyopathy | 2017-02-09 | criteria provided, single submitter | clinical testing | ACMG score unknown significance |
Labcorp Genetics |
RCV000795758 | SCV000935231 | uncertain significance | Loeys-Dietz syndrome 2 | 2023-04-17 | criteria provided, single submitter | clinical testing | This variant is present in population databases (rs375619307, gnomAD 0.006%). This sequence change replaces leucine, which is neutral and non-polar, with phenylalanine, which is neutral and non-polar, at codon 124 of the TMPO protein (p.Leu124Phe). This variant has not been reported in the literature in individuals affected with TMPO-related conditions. 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. An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be disruptive. ClinVar contains an entry for this variant (Variation ID: 427956). |