Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000672477 | SCV000797584 | pathogenic | Fanconi anemia complementation group A | 2018-01-31 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002532125 | SCV003481404 | pathogenic | Fanconi anemia | 2022-11-17 | 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 disrupt the consensus splice site. ClinVar contains an entry for this variant (Variation ID: 556465). This variant is also known as IVS14 + 1G>C. Disruption of this splice site has been observed in individuals with Fanconi anemia (PMID: 9399890, 24584348). This variant is not present in population databases (gnomAD no frequency). This sequence change affects a donor splice site in intron 14 of the FANCA gene. It is expected to disrupt RNA splicing. Variants that disrupt the donor or acceptor splice site typically lead to a loss of protein function (PMID: 16199547), and loss-of-function variants in FANCA are known to be pathogenic (PMID: 19367192). |
Baylor Genetics | RCV000672477 | SCV004196645 | pathogenic | Fanconi anemia complementation group A | 2022-01-11 | criteria provided, single submitter | clinical testing | |
Leiden Open Variation Database | RCV000672477 | SCV001425787 | pathogenic | Fanconi anemia complementation group A | 2020-02-28 | no assertion criteria provided | curation | Curator: Arleen D. Auerbach. Submitter to LOVD: Arleen D. Auerbach. |