Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001723807 | SCV001949923 | likely benign | Li-Fraumeni syndrome 1 | 2021-08-02 | reviewed by expert panel | curation | Transactivation assays show supertransactivation function according to Kato, et al. and there is no evidence of a dominant negative effect or loss of function according to Giacomelli, et al. (BS3; PMID: 12826609, 30224644). . This variant has a BayesDel score < 0.16 and Align GVGD (Zebrafish) is Class C0 or Class C15 (BP4). This variant has been observed in 4 60+ year old females without a cancer diagnosis (BS2_Supporting; internal laboratory contributors). This variant is absent in the gnomAD cohort (PM2_Supporting; http://gnomad.broadinstitute.org) In summary, NM_000546.5(TP53):c.28G>C (p.Val10Leu) meets criteria to be classified as likely benign for Li-Fraumeni syndrome. ACMG/AMP criteria applied, as specified by the TP53 Variant Curation Expert Panel: BS3, PM2_Supporting, BP4, BS2_Supporting. |
Ambry Genetics | RCV000220427 | SCV000275220 | likely benign | Hereditary cancer-predisposing syndrome | 2020-03-23 | criteria provided, single submitter | clinical testing | This alteration is classified as likely benign based on a combination of the following: seen in unaffected individuals, population frequency, intact protein function, lack of segregation with disease, co-occurrence, RNA analysis, in silico models, amino acid conservation, lack of disease association in case-control studies, and/or the mechanism of disease or impacted region is inconsistent with a known cause of pathogenicity. |
Color Diagnostics, |
RCV000220427 | SCV000904841 | uncertain significance | Hereditary cancer-predisposing syndrome | 2018-09-13 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000791760 | SCV000931022 | likely benign | Li-Fraumeni syndrome | 2023-03-28 | criteria provided, single submitter | clinical testing |