Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001547732 | SCV001767509 | uncertain significance | not provided | 2020-06-05 | criteria provided, single submitter | clinical testing | Not observed at a significant frequency in large population cohorts (Lek et al., 2016); In silico analysis supports a deleterious effect on splicing; This variant is associated with the following publications: (PMID: 23528209, 28236338, 30916489) |
Invitae | RCV001547732 | SCV004293946 | pathogenic | not provided | 2023-04-07 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Studies have shown that this variant is associated with altered splicing resulting in multiple RNA products (PMID: 23528209). ClinVar contains an entry for this variant (Variation ID: 1188072). This variant has been observed in individual(s) with congenital ichthyosis (PMID: 23528209). It has also been observed to segregate with disease in related individuals. This variant is present in population databases (no rsID available, gnomAD 0.0009%). This sequence change affects codon 1152 of the ABCA12 mRNA. It is a 'silent' change, meaning that it does not change the encoded amino acid sequence of the ABCA12 protein. |