Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000545258 | SCV000644334 | uncertain significance | Tuberous sclerosis 2 | 2024-10-15 | criteria provided, single submitter | clinical testing | This sequence change replaces proline, which is neutral and non-polar, with leucine, which is neutral and non-polar, at codon 768 of the TSC2 protein (p.Pro768Leu). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with TSC2-related conditions. ClinVar contains an entry for this variant (Variation ID: 467933). Invitae Evidence Modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) indicates that this missense variant is not expected to disrupt TSC2 protein function with a negative predictive value of 95%. 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 | RCV002448717 | SCV002734875 | uncertain significance | Hereditary cancer-predisposing syndrome | 2024-12-10 | criteria provided, single submitter | clinical testing | The p.P768L variant (also known as c.2303C>T), located in coding exon 20 of the TSC2 gene, results from a C to T substitution at nucleotide position 2303. The proline at codon 768 is replaced by leucine, an amino acid with similar properties. This amino acid position is highly conserved in available vertebrate species. In addition, this alteration is predicted to be deleterious by in silico analysis. Based on the available evidence, the clinical significance of this variant remains unclear. |
Baylor Genetics | RCV004568831 | SCV005054440 | uncertain significance | Isolated focal cortical dysplasia type II | 2024-03-15 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV005018945 | SCV005645024 | uncertain significance | Lymphangiomyomatosis; Isolated focal cortical dysplasia type II; Tuberous sclerosis 2 | 2024-02-03 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV004735611 | SCV005357575 | uncertain significance | TSC2-related disorder | 2024-08-24 | no assertion criteria provided | clinical testing | The TSC2 c.2303C>T variant is predicted to result in the amino acid substitution p.Pro768Leu. To our knowledge, this variant has not been reported in the literature or in a large population database, indicating this variant is rare. This variant is interpreted as uncertain in ClinVar (https://www.ncbi.nlm.nih.gov/clinvar/variation/467933/). At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |