Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Center For Human Genetics And Laboratory Diagnostics, |
RCV001726715 | SCV001961038 | uncertain significance | Kleefstra syndrome 1 | 2021-07-26 | criteria provided, single submitter | clinical testing | |
Molecular Genetics Lab, |
RCV001726715 | SCV004697667 | pathogenic | Kleefstra syndrome 1 | criteria provided, single submitter | clinical testing | ||
Laboratory of Genetics, |
RCV001726715 | SCV005045854 | pathogenic | Kleefstra syndrome 1 | criteria provided, single submitter | curation | ||
Gene |
RCV004762170 | SCV005372188 | likely pathogenic | not provided | 2024-01-10 | criteria provided, single submitter | clinical testing | Identified in a patient with Kleefstra syndrome, however, detailed clinical and segregation information were not provided (PMID: 35904121); Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports a deleterious effect on splicing; This variant is associated with the following publications: (PMID: 35904121) |
Prevention |
RCV003941106 | SCV004759465 | uncertain significance | EHMT1-related disorder | 2023-11-21 | no assertion criteria provided | clinical testing | The EHMT1 c.3459C>T variant is not predicted to result in an amino acid change (p.=). This variant is predicted to impact splicing and has been reported in an individual with Kleefstra syndrome (Supplementary Table 1, Levy et al. 2022. PubMed ID: 35904121). This variant has not been reported in a large population database, indicating this variant is rare. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |