Total submissions: 9
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000466964 | SCV000547771 | pathogenic | Fanconi anemia | 2025-01-27 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with tryptophan, which is neutral and slightly polar, at codon 951 of the FANCA protein (p.Arg951Trp). This variant is present in population databases (rs755546887, gnomAD 0.009%). This missense change has been observed in individual(s) with Fanconi anemia (PMID: 17924555, 22778927, 24584348, 26799702). In at least one individual the data is consistent with being in trans (on the opposite chromosome) from a pathogenic variant. ClinVar contains an entry for this variant (Variation ID: 408196). Invitae Evidence Modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) indicates that this missense variant is expected to disrupt FANCA protein function with a positive predictive value of 95%. Experimental studies have shown that this missense change affects FANCA function (PMID: 24349332). For these reasons, this variant has been classified as Pathogenic. |
Revvity Omics, |
RCV000669024 | SCV002022303 | pathogenic | Fanconi anemia complementation group A | 2020-04-01 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV000669024 | SCV002784878 | likely pathogenic | Fanconi anemia complementation group A | 2021-07-05 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV000669024 | SCV004196017 | pathogenic | Fanconi anemia complementation group A | 2024-03-26 | criteria provided, single submitter | clinical testing | |
Genomic Medicine Center of Excellence, |
RCV000669024 | SCV005016611 | likely pathogenic | Fanconi anemia complementation group A | 2024-03-14 | criteria provided, single submitter | clinical testing | |
Counsyl | RCV000669024 | SCV000793720 | likely pathogenic | Fanconi anemia complementation group A | 2017-08-29 | no assertion criteria provided | clinical testing | |
Leiden Open Variation Database | RCV000669024 | SCV001425696 | pathogenic | Fanconi anemia complementation group A | 2020-02-28 | no assertion criteria provided | curation | Curator: Arleen D. Auerbach. Submitters to LOVD: Arleen D. Auerbach, Daniela Pilonetto, Johan de Winter, Sue Richards. |
Gene |
RCV000669024 | SCV001737415 | not provided | Fanconi anemia complementation group A | no assertion provided | literature only | Associated with slower hematologic disease progression | |
Natera, |
RCV000466964 | SCV002092574 | pathogenic | Fanconi anemia | 2021-02-20 | no assertion criteria provided | clinical testing |