Total submissions: 2
| Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
|---|---|---|---|---|---|---|---|---|
| Gene |
RCV004721158 | SCV005328339 | pathogenic | not provided | 2022-05-27 | criteria provided, single submitter | clinical testing | Published functional studies demonstrate a damaging effect; specifically showing the p.E445X variant causes a truncated protein with high stability, but reduced transactivation with dominant negative effect (Angelozzi et al., 2022); Nonsense variant predicted to result in protein truncation as the last 30 amino acids are lost, although loss-of-function variants have not been reported downstream of this position in the protein; Not observed in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 35232796) |
| OMIM | RCV003152402 | SCV003840993 | pathogenic | Coffin-Siris syndrome 10 | 2023-02-02 | no assertion criteria provided | literature only |