Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000578837 | SCV000681109 | likely pathogenic | not provided | 2017-11-15 | criteria provided, single submitter | clinical testing | The Q766X variant in the ABCA12 gene has not been reported previously as a pathogenic variant, nor as a benign variant, to our knowledge. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The Q766X variant is not observed in large population cohorts (Lek et al., 2016). We interpret Q766X as a likely pathogenic variant. |
Invitae | RCV000578837 | SCV004482921 | pathogenic | not provided | 2023-02-14 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Gln766*) in the ABCA12 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in ABCA12 are known to be pathogenic (PMID: 20672373). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with ABCA12-related conditions. ClinVar contains an entry for this variant (Variation ID: 489123). For these reasons, this variant has been classified as Pathogenic. |