Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Genomic Medicine Center of Excellence, |
RCV001420431 | SCV001622563 | likely pathogenic | Anemia, nonspherocytic hemolytic, due to G6PD deficiency | 2021-04-28 | criteria provided, single submitter | research | |
Dunham Lab, |
RCV001420431 | SCV002599163 | likely pathogenic | Anemia, nonspherocytic hemolytic, due to G6PD deficiency | 2022-08-12 | criteria provided, single submitter | curation | Variant found in hemizygote with G6PD deficiency (PP4); inherited from mother (PP1). Decreased activity in red blood cells of hemizygote (PS3). Not observed in gnomAD (PM2). Post_P 0.975 (odds of pathogenicity 350.3, Prior_P 0.1). |
Labcorp Genetics |
RCV001420431 | SCV004633034 | uncertain significance | Anemia, nonspherocytic hemolytic, due to G6PD deficiency | 2023-05-25 | criteria provided, single submitter | clinical testing | 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. ClinVar contains an entry for this variant (Variation ID: 1098498). This sequence change replaces threonine, which is neutral and polar, with isoleucine, which is neutral and non-polar, at codon 51 of the G6PD protein (p.Thr51Ile). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with G6PD-related conditions (PMID: 33708821, 34272389). This variant is also known as c.242C>T p.Thr81Ile. 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 G6PD protein function. Experimental studies have shown that this missense change does not substantially affect G6PD function (PMID: 31489982, 33708821). |