Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000409455 | SCV000485660 | pathogenic | Fanconi anemia complementation group A | 2016-08-16 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001865263 | SCV002159720 | pathogenic | Fanconi anemia | 2022-06-08 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 370361). This premature translational stop signal has been observed in individual(s) with clinical features of Fanconi anemia (PMID: 15522956). This variant is not present in population databases (gnomAD no frequency). This sequence change creates a premature translational stop signal (p.Gln99*) in the FANCA gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in FANCA are known to be pathogenic (PMID: 19367192). |
Gene |
RCV003126717 | SCV003803212 | pathogenic | not provided | 2022-08-08 | criteria provided, single submitter | clinical testing | Nonsense variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 25525159, 31589614, Sipe_2022, 31543367, 33718801, 15522956) |
Leiden Open Variation Database | RCV000409455 | SCV001425762 | pathogenic | Fanconi anemia complementation group A | 2020-02-28 | no assertion criteria provided | curation | Curator: Arleen D. Auerbach. Submitters to LOVD: Arleen D. Auerbach, Sue Richards. |