Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002847393 | SCV003227389 | pathogenic | Fanconi anemia | 2022-08-05 | criteria provided, single submitter | clinical testing | This variant has not been reported in the literature in individuals affected with FANCA-related conditions. This variant is not present in population databases (gnomAD no frequency). This sequence change creates a premature translational stop signal (p.Ile1101Thrfs*14) 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). For these reasons, this variant has been classified as Pathogenic. |
Prevention |
RCV004744458 | SCV005341815 | likely pathogenic | FANCA-related disorder | 2024-04-18 | no assertion criteria provided | clinical testing | The FANCA c.3302_3308del7 variant is predicted to result in a frameshift and premature protein termination (p.Ile1101Thrfs*14). To our knowledge, this variant has not been reported in the literature or in a large population database, indicating this variant is rare. Frameshift variants in FANCA are expected to be pathogenic. This variant is interpreted as likely pathogenic. |