Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000579307 | SCV000339946 | pathogenic | not provided | 2016-02-23 | criteria provided, single submitter | clinical testing | |
Gene |
RCV000579307 | SCV000680704 | pathogenic | not provided | 2022-01-21 | criteria provided, single submitter | clinical testing | Not observed at significant frequency in large population cohorts (gnomAD); 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; Reported in ClinVar (ClinVar Variant ID# 286497; ClinVar); This variant is associated with the following publications: (PMID: 25525159, 20513134, 10729292, 26443184, 23022073, 29453956, 20179744, 31758797, 32756486, 32639332, 27408751, 28559085, 26582918) |
Labcorp Genetics |
RCV000579307 | SCV001591749 | pathogenic | not provided | 2024-11-16 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg785*) in the COL2A1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in COL2A1 are known to be pathogenic (PMID: 20179744). This variant is not present in population databases (gnomAD no frequency). This premature translational stop signal has been observed in individuals with autosomal dominant Stickler syndrome (PMID: 10729292, 28559085). It has also been observed to segregate with disease in related individuals. This variant is also known as 22314 C-T, R585X. ClinVar contains an entry for this variant (Variation ID: 286497). For these reasons, this variant has been classified as Pathogenic. |
Division of Genomic Medicine, |
RCV003380537 | SCV004098662 | pathogenic | Stickler syndrome type 1 | 2023-10-20 | criteria provided, single submitter | clinical testing | |
Juno Genomics, |
RCV004796151 | SCV005416076 | pathogenic | Stickler syndrome type 1; Stickler syndrome, type I, nonsyndromic ocular | criteria provided, single submitter | clinical testing | PM2_Supporting+PVS1+PS4_Moderate+PP1_Strong+PP4 |