Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001857336 | SCV002288127 | uncertain significance | not provided | 2023-12-27 | 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 317 of the CFI protein (p.Arg317Trp). This variant is present in population databases (rs121964917, gnomAD 0.02%). This missense change has been observed in individual(s) with atypical hemolytic uremic syndrome (PMID: 16621965, 34169201). This variant is also known as R299W. ClinVar contains an entry for this variant (Variation ID: 12125). 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 not expected to disrupt CFI protein function with a negative predictive value of 80%. Experimental studies are conflicting or provide insufficient evidence to determine the effect of this variant on CFI function (PMID: 17597211, 19877009). 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. |
Mayo Clinic Laboratories, |
RCV001857336 | SCV002542094 | uncertain significance | not provided | 2021-05-20 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV002496331 | SCV002787195 | uncertain significance | Atypical hemolytic-uremic syndrome with I factor anomaly; Age related macular degeneration 13; Factor I deficiency | 2021-07-14 | criteria provided, single submitter | clinical testing | |
OMIM | RCV000012908 | SCV000033149 | risk factor | Atypical hemolytic-uremic syndrome with I factor anomaly | 2006-08-15 | no assertion criteria provided | literature only |