Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000818422 | SCV000959034 | uncertain significance | Li-Fraumeni syndrome | 2018-12-16 | criteria provided, single submitter | clinical testing | 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. Nucleotide substitutions within the consensus splice site are a relatively common cause of aberrant splicing (PMID: 17576681, 9536098). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site, but this prediction has not been confirmed by published transcriptional studies. This variant has not been reported in the literature in individuals with TP53-related conditions. This variant is not present in population databases (ExAC no frequency). This sequence change falls in intron 9 of the TP53 gene. It does not directly change the encoded amino acid sequence of the TP53 protein, but it affects a nucleotide within the consensus splice site of the intron. |
Mendelics | RCV000989704 | SCV001140243 | uncertain significance | Squamous cell carcinoma of the head and neck | 2019-05-28 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV001019914 | SCV001181328 | uncertain significance | Hereditary cancer-predisposing syndrome | 2020-10-19 | criteria provided, single submitter | clinical testing | The c.993+5T>G intronic variant results from a T to G substitution 5 nucleotides after coding exon 8 in the TP53 gene. This nucleotide position is poorly conserved in available vertebrate species. In silico splice site analysis predicts that this alteration will not have any significant effect on splicing; however, direct evidence is insufficient at this time (Ambry internal data). Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Genome- |
RCV002290464 | SCV002582844 | uncertain significance | Li-Fraumeni syndrome 1 | 2022-06-18 | criteria provided, single submitter | clinical testing | |
Genome- |
RCV001019914 | SCV002583226 | uncertain significance | Hereditary cancer-predisposing syndrome | 2022-06-18 | criteria provided, single submitter | clinical testing |