Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002524688 | SCV002960445 | pathogenic | RASopathy | 2022-07-25 | criteria provided, single submitter | clinical testing | Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt KRAS protein function. This sequence change replaces alanine, which is neutral and non-polar, with valine, which is neutral and non-polar, at codon 146 of the KRAS protein (p.Ala146Val). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individuals with mosaic RASopathy (PMID: 26970110, 30891959). ClinVar contains an entry for this variant (Variation ID: 375962). Experimental studies have shown that this missense change affects KRAS function (PMID: 30448735). This variant disrupts the p.Ala146 amino acid residue in KRAS. Other variant(s) that disrupt this residue have been determined to be pathogenic (Invitae). This suggests that this residue is clinically significant, and that variants that disrupt this residue are likely to be disease-causing. For these reasons, this variant has been classified as Pathogenic. |
Institute of Human Genetics, |
RCV003488585 | SCV004239231 | uncertain significance | not provided | 2023-11-14 | criteria provided, single submitter | clinical testing | |
Genomic Medicine Center of Excellence, |
RCV004760489 | SCV005373897 | likely pathogenic | Familial pancreatic carcinoma | 2024-09-22 | criteria provided, single submitter | clinical testing | |
OMIM | RCV000791299 | SCV000930609 | pathogenic | OCULOECTODERMAL SYNDROME, SOMATIC | 2022-03-09 | no assertion criteria provided | literature only | |
Gene |
RCV001839452 | SCV002099551 | not provided | Encephalocraniocutaneous lipomatosis | no assertion provided | literature only | ||
Laboratory of Urology, |
RCV003332167 | SCV004040498 | pathogenic | Malignant tumor of urinary bladder | no assertion criteria provided | research |