Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
St. |
RCV003444457 | SCV004171529 | pathogenic | Fanconi anemia complementation group A | 2023-11-01 | criteria provided, single submitter | clinical testing | The FANCA c.1626+967_2015-679del variant is a gross deletion of the genomic region encompassing exons 18-22 of the FANCA gene. The 5’ end is likely confined to intron 17. The 3’ end of this event is likely confined to intron 22. This deletion is predicted to cause a frameshift and premature protein truncation. Deletions involving exons 18-22 FANCA have been reported in the literature in individuals with Fanconi anemia (PMID: 15643609, 17924555, 19367192, 21273304, 28717661, 29098742, 34585473). Loss-of-function variants in FANCA are known to be pathogenic (PMID: 19367192). In summary, this variant meets criteria to be classified as pathogenic. |