Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001298698 | SCV001487761 | uncertain significance | Torsion dystonia 6 | 2023-08-10 | criteria provided, single submitter | clinical testing | ClinVar contains an entry for this variant (Variation ID: 1002296). This variant has not been reported in the literature in individuals affected with THAP1-related conditions. This variant is present in population databases (rs761376417, gnomAD 0.03%). This sequence change replaces alanine, which is neutral and non-polar, with proline, which is neutral and non-polar, at codon 115 of the THAP1 protein (p.Ala115Pro). 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 tolerated. |
Gene |
RCV001587326 | SCV001826549 | likely benign | not provided | 2021-04-08 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV004036114 | SCV004965806 | uncertain significance | Inborn genetic diseases | 2024-01-30 | criteria provided, single submitter | clinical testing | The c.343G>C (p.A115P) alteration is located in exon 3 (coding exon 3) of the THAP1 gene. This alteration results from a G to C substitution at nucleotide position 343, causing the alanine (A) at amino acid position 115 to be replaced by a proline (P). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |