Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clinical Genomics Laboratory, |
RCV004556009 | SCV005045163 | likely pathogenic | Marshall syndrome; Stickler syndrome type 2 | 2023-11-29 | criteria provided, single submitter | clinical testing | The COL11A1 c.1951C>T (p.Arg651Ter) variant, to our knowledge, has not been reported in the medical literature and is absent from the general population (gnomAD v.2.1.1), indicating it is not a common variant. This variant causes a premature termination codon, which is predicted to lead to nonsense mediated decay. Additionally, other variants that introduce a premature termination codon in this region are described as pathogenic in the ClinVar database (Variation IDs: 1455649, 2128713, 2131680). Based on available information and the ACMG/AMP guidelines for variant interpretation (Richards S et al., PMID: 25741868), this variant is classified as likely pathogenic. |
Labcorp Genetics |
RCV005100829 | SCV005797806 | pathogenic | not provided | 2024-02-04 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg651*) in the COL11A1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in COL11A1 are known to be pathogenic (PMID: 20513134, 21035103, 23922384, 25240749, 32427345, 32756486). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with COL11A1-related conditions. For these reasons, this variant has been classified as Pathogenic. |
Prevention |
RCV004736693 | SCV005342458 | likely pathogenic | COL11A1-related disorder | 2024-08-29 | no assertion criteria provided | clinical testing | The COL11A1 c.1951C>T variant is predicted to result in premature protein termination (p.Arg651*). To our knowledge, this variant has not been reported in a large population database, indicating this variant is rare. Nonsense variants in COL11A1 are expected to be pathogenic. This variant is interpreted as likely pathogenic. |