Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002651608 | SCV003524922 | pathogenic | not provided | 2022-09-29 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with cysteine, which is neutral and slightly polar, at codon 158 of the IHH protein (p.Arg158Cys). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with autosomal dominant brachydactyly (PMID: 19464397). It has also been observed to segregate with disease in related individuals. Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt IHH protein function. For these reasons, this variant has been classified as Pathogenic. |
Revvity Omics, |
RCV002651608 | SCV003813346 | uncertain significance | not provided | 2019-04-12 | criteria provided, single submitter | clinical testing |