Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV001813168 | SCV001474066 | uncertain significance | not provided | 2020-04-03 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001813168 | SCV002966371 | likely pathogenic | not provided | 2025-01-08 | criteria provided, single submitter | clinical testing | This sequence change replaces serine, which is neutral and polar, with proline, which is neutral and non-polar, at codon 53 of the IFNAR2 protein (p.Ser53Pro). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with IFNAR2-related conditions (PMID: 35442417). It has also been observed to segregate with disease in related individuals. ClinVar contains an entry for this variant (Variation ID: 994273). An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be disruptive. Experimental studies have shown that this missense change affects IFNAR2 function (PMID: 35442417). In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. |
OMIM | RCV002284216 | SCV002573544 | pathogenic | Immunodeficiency 45 | 2024-03-01 | no assertion criteria provided | literature only |