Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000695507 | SCV000824013 | pathogenic | Autoimmune lymphoproliferative syndrome type 1 | 2022-02-21 | criteria provided, single submitter | clinical testing | This variant is not present in population databases (gnomAD no frequency). For these reasons, this variant has been classified as Pathogenic. Studies have shown that disruption of this splice site results in skipping of exon 7 and introduces a premature termination codon (PMID: 15459303). The resulting mRNA is expected to undergo nonsense-mediated decay. ClinVar contains an entry for this variant (Variation ID: 573761). Disruption of this splice site has been observed in individuals with autosomal dominant autoimmune lymphoproliferative syndrome (ALPS) (PMID: 9533447, 15459303, 22237435). This sequence change affects a donor splice site in intron 7 of the FAS gene. RNA analysis indicates that disruption of this splice site induces altered splicing and may result in an absent or disrupted protein product. |
Fulgent Genetics, |
RCV000695507 | SCV000893852 | likely pathogenic | Autoimmune lymphoproliferative syndrome type 1 | 2021-07-13 | criteria provided, single submitter | clinical testing |