Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000255106 | SCV000321321 | pathogenic | not provided | 2023-04-20 | criteria provided, single submitter | clinical testing | Observed with a pathogenic variant on the opposite allele (in trans) in unrelated patients with Harlequin ichthyosis referred for genetic testing at GeneDx and reported in the published literature (Thomas et al., 2006); 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: 22344438, 25525159, 20981092, 16902423) |
Genomic Medicine Center of Excellence, |
RCV003989509 | SCV004807345 | pathogenic | Autosomal recessive congenital ichthyosis 4B | 2024-03-26 | criteria provided, single submitter | clinical testing |