Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Fulgent Genetics, |
RCV001268955 | SCV005664067 | likely pathogenic | Immunodeficiency 28 | 2024-04-25 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001268955 | SCV005698233 | likely pathogenic | Immunodeficiency 28 | 2024-08-04 | criteria provided, single submitter | clinical testing | This sequence change affects the initiator methionine of the IFNGR2 mRNA. The next in-frame methionine is located at codon 80. This variant is present in population databases (no rsID available, gnomAD 0.007%). Disruption of the initiator codon has been observed in individual(s) with clinical features of mendelian susceptibility to mycobacterial disease (PMID: 31222290). It has also been observed to segregate with disease in related individuals. ClinVar contains an entry for this variant (Variation ID: 987732). Algorithms developed to predict the effect of variants on gene product structure and function are not available or were not evaluated for this variant. Experimental studies have shown that disruption of the initiator codon affects IFNGR2 function (PMID: 31222290). 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 | RCV001268955 | SCV001448209 | pathogenic | Immunodeficiency 28 | 2021-10-08 | no assertion criteria provided | literature only |