Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003037444 | SCV003441198 | pathogenic | Hyper-IgM syndrome type 2 | 2023-10-13 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Tyr31*) in the AICDA gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in AICDA are known to be pathogenic (PMID: 11007475). This variant is not present in population databases (gnomAD no frequency). This premature translational stop signal has been observed in individual(s) with autosomal recessive Hyper IgM syndrome (PMID: 16964591). ClinVar contains an entry for this variant (Variation ID: 2137297). For these reasons, this variant has been classified as Pathogenic. |