Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000633360 | SCV000754582 | uncertain significance | Li-Fraumeni syndrome | 2023-06-17 | criteria provided, single submitter | clinical testing | This missense change has been observed in individual(s) with clinical features of TP53-related conditions (Invitae). ClinVar contains an entry for this variant (Variation ID: 528254). Advanced modeling performed at Invitae incorporating data from internal and/or published experimental studies (PMID: 12826609, 29979965, 30224644) indicates that this missense variant is expected to disrupt TP53 function. Experimental studies have shown that this missense change affects TP53 function (PMID: 12826609, 29979965, 30224644). This variant disrupts the p.Ile251 amino acid residue in TP53. Other variant(s) that disrupt this residue have been determined to be pathogenic (PMID: 12826609, 21305319, 27501770, 29979965, 30224644). This suggests that this residue is clinically significant, and that variants that disrupt this residue are likely to be disease-causing. 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. This sequence change replaces isoleucine, which is neutral and non-polar, with asparagine, which is neutral and polar, at codon 251 of the TP53 protein (p.Ile251Asn). This variant is not present in population databases (gnomAD no frequency). |
All of Us Research Program, |
RCV000633360 | SCV004827010 | uncertain significance | Li-Fraumeni syndrome | 2023-04-27 | criteria provided, single submitter | clinical testing | |
Myriad Genetics, |
RCV004025427 | SCV004932388 | likely pathogenic | Li-Fraumeni syndrome 1 | 2024-02-16 | criteria provided, single submitter | clinical testing | This variant is considered likely pathogenic. Functional studies indicate this variant impacts protein function [PMID: 29979965]. This variant is expected to disrupt protein structure [Myriad internal data]. |
Cancer Genetics Lab, |
RCV003228966 | SCV003926524 | pathogenic | Acute myeloid leukemia | no assertion criteria provided | clinical testing |