Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Fulgent Genetics, |
RCV001849649 | SCV005678355 | likely pathogenic | Asphyxiating thoracic dystrophy 3 | 2024-01-04 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV005057675 | SCV005715626 | uncertain significance | Jeune thoracic dystrophy | 2024-12-31 | criteria provided, single submitter | clinical testing | This sequence change replaces tyrosine, which is neutral and polar, with cysteine, which is neutral and slightly polar, at codon 3213 of the DYNC2H1 protein (p.Tyr3213Cys). This variant is present in population databases (no rsID available, gnomAD 0.0008%). This missense change has been observed in individual(s) with short-rib thoracic dysplasia (PMID: 30655312). ClinVar contains an entry for this variant (Variation ID: 1344648). Invitae Evidence Modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) indicates that this missense variant is expected to disrupt DYNC2H1 protein function with a positive predictive value of 95%. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
Yale Center for Mendelian Genomics, |
RCV001849649 | SCV002106571 | pathogenic | Asphyxiating thoracic dystrophy 3 | 2019-01-17 | no assertion criteria provided | literature only |