Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000005430 | SCV002108005 | pathogenic | Hyper-IgM syndrome type 2 | 2022-08-09 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. ClinVar contains an entry for this variant (Variation ID: 5123). This premature translational stop signal has been observed in individual(s) with autosomal recessive hyper IgM syndrome (PMID: 11007475, 14962793). This variant is not present in population databases (gnomAD no frequency). This sequence change creates a premature translational stop signal (p.Trp68*) 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). |
Ce |
RCV003389747 | SCV004134383 | pathogenic | not provided | 2023-01-01 | criteria provided, single submitter | clinical testing | AICDA: PVS1, PM2 |
OMIM | RCV000005430 | SCV000025612 | pathogenic | Hyper-IgM syndrome type 2 | 2000-09-01 | no assertion criteria provided | literature only |