Total submissions: 7
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000178223 | SCV000230243 | uncertain significance | not provided | 2014-06-06 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001852208 | SCV002282857 | likely pathogenic | RASopathy | 2024-10-02 | criteria provided, single submitter | clinical testing | This sequence change replaces alanine, which is neutral and non-polar, with threonine, which is neutral and polar, at codon 146 of the KRAS protein (p.Ala146Thr). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with KRAS-related conditions. ClinVar contains an entry for this variant (Variation ID: 197243). Invitae Evidence Modeling incorporating data from in vitro experimental studies (internal data) indicates that this missense variant is expected to disrupt KRAS function with a positive predictive value of 95%. Experimental studies have shown that this missense change affects KRAS function (PMID: 20147967, 20570890, 26110767). This variant disrupts the p.Ala146 amino acid residue in KRAS. Other variant(s) that disrupt this residue have been determined to be pathogenic (internal data). This suggests that this residue is clinically significant, and that variants that disrupt this residue are likely to be disease-causing. In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. |
Pathology Department, |
RCV002227934 | SCV002507273 | uncertain significance | Classic Hodgkin lymphoma | 2021-10-01 | criteria provided, single submitter | research | |
Ce |
RCV000178223 | SCV003917190 | pathogenic | not provided | 2023-01-01 | criteria provided, single submitter | clinical testing | |
Greenwood Genetic Center Diagnostic Laboratories, |
RCV004554743 | SCV005044043 | pathogenic | Cardiofaciocutaneous syndrome 2 | 2024-01-30 | criteria provided, single submitter | clinical testing | PS3, SP4, PM1 |
OMIM | RCV000791298 | SCV000930608 | pathogenic | OCULOECTODERMAL SYNDROME, SOMATIC | 2022-03-09 | no assertion criteria provided | literature only | |
Gene |
RCV001839448 | SCV002099550 | not provided | Encephalocraniocutaneous lipomatosis | no assertion provided | literature only |