Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Invitae | RCV001054571 | SCV001218891 | pathogenic | Fanconi anemia | 2019-03-29 | criteria provided, single submitter | clinical testing | This variant is a deletion of the genomic region encompassing exons 4-6 and part of exon 7 (c.284-1329_704del) of the FANCA gene. It is expected to disrupt RNA splicing and likely results in an absent or disrupted protein product. Similar deletions described as deletion of exons 4-7 have been observed in individuals with clinical features of Fanconi anemia (PMID: 29098742, 26799702, Invitae). Loss-of-function variants in FANCA are known to be pathogenic (PMID: 19367192). For these reasons, this variant has been classified as Pathogenic. |