Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Center of Genomic medicine, |
RCV000857246 | SCV000999833 | likely pathogenic | Imerslund-Grasbeck syndrome | 2019-03-18 | criteria provided, single submitter | clinical testing | This variant was identified in combination with a second variant in trans in the same gene (CUBN - composite heterozygosity) in a patient with proteinuria and glomerulopathy |
Gene |
RCV001776065 | SCV002012659 | uncertain significance | not provided | 2019-08-10 | criteria provided, single submitter | clinical testing | In silico analysis, which includes protein predictors and evolutionary conservation, supports a deleterious effect; Has not been previously published as pathogenic or benign to our knowledge |
Labcorp Genetics |
RCV000857246 | SCV002270977 | uncertain significance | Imerslund-Grasbeck syndrome | 2022-10-31 | criteria provided, single submitter | clinical testing | This sequence change replaces cysteine, which is neutral and slightly polar, with tyrosine, which is neutral and polar, at codon 2032 of the CUBN protein (p.Cys2032Tyr). This variant is present in population databases (rs201720797, gnomAD 0.02%). This variant has not been reported in the literature in individuals affected with CUBN-related conditions. ClinVar contains an entry for this variant (Variation ID: 695032). 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 CUBN protein function. 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. |
Eurofins- |
RCV003236590 | SCV003935130 | likely pathogenic | Proteinuria, chronic benign | 2022-12-01 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV005036241 | SCV005673149 | uncertain significance | Imerslund-Grasbeck syndrome type 1; Proteinuria, chronic benign | 2024-06-11 | criteria provided, single submitter | clinical testing |