Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001862653 | SCV002290610 | uncertain significance | Short-rib thoracic dysplasia 10 with or without polydactyly; Retinitis pigmentosa 71 | 2022-10-13 | criteria provided, single submitter | clinical testing | 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. 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 not expected to disrupt IFT172 protein function. ClinVar contains an entry for this variant (Variation ID: 869114). This missense change has been observed in individual(s) with Bardet-Biedl syndrome (PMID: 32451492). This variant is present in population databases (rs144645349, gnomAD 0.01%). This sequence change replaces histidine, which is basic and polar, with tyrosine, which is neutral and polar, at codon 719 of the IFT172 protein (p.His719Tyr). |
Fulgent Genetics, |
RCV002482157 | SCV002779310 | uncertain significance | Short-rib thoracic dysplasia 10 with or without polydactyly; Retinitis pigmentosa 71; Bardet-Biedl syndrome 20 | 2022-04-05 | criteria provided, single submitter | clinical testing | |
Dept Of Ophthalmology, |
RCV003890231 | SCV004705263 | uncertain significance | Retinal dystrophy | 2023-10-01 | criteria provided, single submitter | research | |
Department of Neurology, |
RCV001257322 | SCV001243222 | pathogenic | Bardet-Biedl syndrome 22 | 2020-03-24 | no assertion criteria provided | case-control | |
OMIM | RCV002508153 | SCV001769495 | pathogenic | Bardet-Biedl syndrome 20 | 2021-08-04 | no assertion criteria provided | literature only | |
Prevention |
RCV004738157 | SCV005351750 | uncertain significance | IFT172-related disorder | 2024-03-25 | no assertion criteria provided | clinical testing | The IFT172 c.2155C>T variant is predicted to result in the amino acid substitution p.His719Tyr. This variant was reported in the compound heterozygous state in an individual with Bardet-Biedl syndrome (Hirano et al. 2020. PubMed ID: 32451492). This variant is reported in 0.016% of alleles in individuals of East Asian descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |