Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001558657 | SCV001780652 | pathogenic | not provided | 2021-09-07 | criteria provided, single submitter | clinical testing | 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; Observed with a second DLL3 variant in an individual with spondylocostal dysostosis in the published literature (Bonafe et al., 2003); This variant is associated with the following publications: (PMID: 12791036, 23496422, 17213840, 25525159) |
Labcorp Genetics |
RCV001558657 | SCV002146403 | pathogenic | not provided | 2024-01-19 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg238*) in the DLL3 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in DLL3 are known to be pathogenic (PMID: 12746394). This variant is present in population databases (rs104894675, gnomAD 0.007%). This premature translational stop signal has been observed in individual(s) with spondylocostal dysostosis (PMID: 12791036). ClinVar contains an entry for this variant (Variation ID: 6833). For these reasons, this variant has been classified as Pathogenic. |
Baylor Genetics | RCV000007235 | SCV004040808 | pathogenic | Spondylocostal dysostosis 1, autosomal recessive | 2023-05-05 | criteria provided, single submitter | clinical testing | |
OMIM | RCV000007235 | SCV000027431 | pathogenic | Spondylocostal dysostosis 1, autosomal recessive | 2003-07-01 | no assertion criteria provided | literature only |